Familial mediterranean fever differential diagnosis: Difference between revisions

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__NOTOC__
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{{Familial mediterranean fever}}
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{{CMG}}; {{AE}} {{Sahar}}
==Overview==
Familial mediterranean fever must be differentiated from other diseases that cause [[fever]], [[fatigue]], [[weight loss]], [[arthralgia]], [[myalgia]], [[rash]] and [[soft tissue]] [[swelling]].
==Differential diagnosis==
Familial mediterranean fever (FMF) should be differentiated from other conditions presenting with [[fever]], [[fatigue]], [[weight loss]], [[arthralgia]], [[myalgia]], [[rash]] and [[soft tissue]] [[swelling]]. The differentials include the following:<ref name="pmid18163139">{{cite journal |vauthors=Ejilemele AA, Nwauche CA, Ejele OA |title=Pattern of abnormal liver enzymes in HIV patients presenting at a Nigerian Tertiary Hospital |journal=Niger Postgrad Med J |volume=14 |issue=4 |pages=306–9 |date=December 2007 |pmid=18163139 |doi= |url=}}</ref><ref name="pmid1579936">{{cite journal |vauthors=Gøransson LG, Omdal R, Husby G |title=[Adult-onset Still's disease. Diagnosis, differential diagnosis and treatment] |language=Norwegian |journal=Tidsskr. Nor. Laegeforen. |volume=112 |issue=9 |pages=1155–5 |date=March 1992 |pmid=1579936 |doi= |url=}}</ref><ref name="pmid21734024">{{cite journal |vauthors=Hatakka A, Klein J, He R, Piper J, Tam E, Walkty A |title=Acute hepatitis as a manifestation of parvovirus B19 infection |journal=J. Clin. Microbiol. |volume=49 |issue=9 |pages=3422–4 |date=September 2011 |pmid=21734024 |pmc=3165617 |doi=10.1128/JCM.00575-11 |url=}}</ref><ref name="pmid25780346">{{cite journal |vauthors=Yaguchi D, Marui N, Matsuo M |title=Three Adult Cases of HPV-B19 Infection with Concomitant Leukopenia and Low Platelet Counts |journal=Clin Med Insights Case Rep |volume=8 |issue= |pages=19–22 |date=2015 |pmid=25780346 |pmc=4345940 |doi=10.4137/CCRep.S18085 |url=}}</ref><ref name="pmid11810534">{{cite journal |vauthors=Díaz F, Collazos J |title=Hepatic dysfunction due to parvovirus B19 infection |journal=J. Infect. Chemother. |volume=6 |issue=1 |pages=63–4 |date=March 2000 |pmid=11810534 |doi=10.1007/s101560000023 |url=}}</ref><ref name="urlwatermark.silverchair.com">{{cite web |url=https://watermark.silverchair.com/22-5-783.pdf?token=AQECAHi208BE49Ooan9kkhW_Ercy7Dm3ZL_9Cf3qfKAc485ysgAAAaUwggGhBgkqhkiG9w0BBwagggGSMIIBjgIBADCCAYcGCSqGSIb3DQEHATAeBglghkgBZQMEAS4wEQQMNepsAzlRiawaxgMCAgEQgIIBWCcwAN4r_jjoL6cD8qOBCgCIgpagHIemSBzmiP_c08L34mY51H7ulShPj9n2yDVBVRols0OgZEIsKVl8kHD_89enHvhFrJOIh8cF7GxZ_AXULME_iReJXr37VWLU5bWFrtZHafAg_cvOdP8mr5u5K8TzGLBtd5VfQb2MGyXZhz-hpKGZbfMr33rJio00VHcFn_aS5wjB-h6fny_rsD4fwLj5xSWNB0_JEYq9g0-mS7y_igwhIZlismSrS4-svrkx46SDLpKqkTrJmImyDBGTj1Qi3INV8KT66j4j6z2wju7sRKvWxQAsoJhN6D8zxx5PQkth3gLDch3wLW_sn9o8q7sBlO25ONobKarrln8nwsS4f-9wHmPs82ZySggX4SgVtrMn6tr3lMVcroGV9fjOIcQQuk_KRq01ErxBdPtynPVbAZhmInGIJ0_LYqvOKplDpNnMHZZaEQYd |title=watermark.silverchair.com |format= |work= |accessdate=}}</ref><ref name="pmid23355575">{{cite journal |vauthors=Shetty RK, Vivek G, Naha K, Bekkam S |title=Right-sided infective endocarditis presenting with purpuric skin rash and cardiac failure in a patient without fever |journal=BMJ Case Rep |volume=2013 |issue= |pages= |date=January 2013 |pmid=23355575 |pmc=3603787 |doi=10.1136/bcr-2012-007841 |url=}}</ref><ref name="pmid23133445">{{cite journal |vauthors=Aucott JN, Crowder LA, Yedlin V, Kortte KB |title=Bull's-Eye and Nontarget Skin Lesions of Lyme Disease: An Internet Survey of Identification of Erythema Migrans |journal=Dermatol Res Pract |volume=2012 |issue= |pages=451727 |date=2012 |pmid=23133445 |pmc=3485866 |doi=10.1155/2012/451727 |url=}}</ref><ref name="pmid21772606">{{cite journal |vauthors=Karaali Z, Baysal B, Poturoglu S, Kendir M |title=Cutaneous manifestations in brucellosis |journal=Indian J Dermatol |volume=56 |issue=3 |pages=339–40 |date=May 2011 |pmid=21772606 |pmc=3132922 |doi=10.4103/0019-5154.82505 |url=}}</ref><ref name="pmid18843212">{{cite journal |vauthors=La Spada E, Micalizzi A, La Spada M, Quartarano P, Nugara G, Soresi M, Affronti M, Montalto G |title=[Abnormal liver function in brucellosis] |language=Italian |journal=Infez Med |volume=16 |issue=3 |pages=148–53 |date=September 2008 |pmid=18843212 |doi= |url=}}</ref><ref name="pmid17235095">{{cite journal |vauthors=French P |title=Syphilis |journal=BMJ |volume=334 |issue=7585 |pages=143–7 |date=January 2007 |pmid=17235095 |pmc=1779891 |doi=10.1136/bmj.39085.518148.BE |url=}}</ref><ref name="urlSyphilis: Review with Emphasis on Clinical, Epidemiologic, and Some Biologic Features">{{cite web |url=http://cmr.asm.org/content/12/2/187.full |title=Syphilis: Review with Emphasis on Clinical, Epidemiologic, and Some Biologic Features |format= |work= |accessdate=}}</ref><ref name="pmid24700957">{{cite journal |vauthors=Baveja S, Garg S, Rajdeo A |title=Syphilitic hepatitis: an uncommon manifestation of a common disease |journal=Indian J Dermatol |volume=59 |issue=2 |pages=209 |date=March 2014 |pmid=24700957 |pmc=3969699 |doi=10.4103/0019-5154.127711 |url=}}</ref><ref name="pmid1600010">{{cite journal |vauthors=Mawhorter SD, Effron D, Blinkhorn R, Spagnuolo PJ |title=Cutaneous manifestations of toxoplasmosis |journal=Clin. Infect. Dis. |volume=14 |issue=5 |pages=1084–8 |date=May 1992 |pmid=1600010 |doi= |url=}}</ref><ref name="pmid24662942">{{cite journal |vauthors=Flegr J, Prandota J, Sovičková M, Israili ZH |title=Toxoplasmosis--a global threat. Correlation of latent toxoplasmosis with specific disease burden in a set of 88 countries |journal=PLoS ONE |volume=9 |issue=3 |pages=e90203 |date=2014 |pmid=24662942 |pmc=3963851 |doi=10.1371/journal.pone.0090203 |url=}}</ref><ref name="pmid21887062">{{cite journal |vauthors=Furtado JM, Smith JR, Belfort R, Gattey D, Winthrop KL |title=Toxoplasmosis: a global threat |journal=J Glob Infect Dis |volume=3 |issue=3 |pages=281–4 |date=July 2011 |pmid=21887062 |pmc=3162817 |doi=10.4103/0974-777X.83536 |url=}}</ref><ref name="pmid10808314">{{cite journal |vauthors=Ripert C |title=[Reactive hypereosinophilia in parasitic diseases] |language=French |journal=Rev Prat |volume=50 |issue=6 |pages=602–7 |date=March 2000 |pmid=10808314 |doi= |url=}}</ref><ref name="pmid21569516">{{cite journal |vauthors=Alvarado-Esquivel C, Torres-Berumen JL, Estrada-Martínez S, Liesenfeld O, Mercado-Suarez MF |title=Toxoplasma gondii infection and liver disease: a case-control study in a northern Mexican population |journal=Parasit Vectors |volume=4 |issue= |pages=75 |date=May 2011 |pmid=21569516 |pmc=3105944 |doi=10.1186/1756-3305-4-75 |url=}}</ref><ref name="pmid5339237">{{cite journal |vauthors=Han T, Stutzman L |title=Mode of spread in patients with localized malignant lymphoma |journal=Arch. Intern. Med. |volume=120 |issue=1 |pages=1–7 |date=July 1967 |pmid=5339237 |doi= |url=}}</ref><ref name="pmid23071471">{{cite journal |vauthors=Saeed-Abdul-Rahman I, Al-Amri AM |title=Castleman disease |journal=Korean J Hematol |volume=47 |issue=3 |pages=163–77 |date=September 2012 |pmid=23071471 |pmc=3464333 |doi=10.5045/kjh.2012.47.3.163 |url=}}</ref><ref name="pmid230714712">{{cite journal |vauthors=Saeed-Abdul-Rahman I, Al-Amri AM |title=Castleman disease |journal=Korean J Hematol |volume=47 |issue=3 |pages=163–77 |date=September 2012 |pmid=23071471 |pmc=3464333 |doi=10.5045/kjh.2012.47.3.163 |url=}}</ref><ref name="pmid21063526">{{cite journal |vauthors=Papadavid E, Panayiotides I, Dalamaga M, Katoulis A, Economopoulos T, Stavrianeas N |title=Cutaneous involvement in angioimmunoblastic T-cell lymphoma |journal=Indian J Dermatol |volume=55 |issue=3 |pages=279–80 |date=2010 |pmid=21063526 |pmc=2965920 |doi=10.4103/0019-5154.70704 |url=}}</ref><ref name="pmid26120552">{{cite journal |vauthors=Brockow K, Przybilla B, Aberer W, Bircher AJ, Brehler R, Dickel H, Fuchs T, Jakob T, Lange L, Pfützner W, Mockenhaupt M, Ott H, Pfaar O, Ring J, Sachs B, Sitter H, Trautmann A, Treudler R, Wedi B, Worm M, Wurpts G, Zuberbier T, Merk HF |title=Guideline for the diagnosis of drug hypersensitivity reactions: S2K-Guideline of the German Society for Allergology and Clinical Immunology (DGAKI) and the German Dermatological Society (DDG) in collaboration with the Association of German Allergologists (AeDA), the German Society for Pediatric Allergology and Environmental Medicine (GPA), the German Contact Dermatitis Research Group (DKG), the Swiss Society for Allergy and Immunology (SGAI), the Austrian Society for Allergology and Immunology (ÖGAI), the German Academy of Allergology and Environmental Medicine (DAAU), the German Center for Documentation of Severe Skin Reactions and the German Federal Institute for Drugs and Medical Products (BfArM) |journal=Allergo J Int |volume=24 |issue=3 |pages=94–105 |date=2015 |pmid=26120552 |pmc=4479479 |doi=10.1007/s40629-015-0052-6 |url=}}</ref><ref name="pmid15745878">{{cite journal |vauthors=Medlej-Hashim M, Loiselet J, Lefranc G, Mégarbané A |title=[Familial Mediterranean Fever (FMF): from diagnosis to treatment] |language=French |journal=Sante |volume=14 |issue=4 |pages=261–6 |date=2004 |pmid=15745878 |doi= |url=}}</ref><ref name="pmid27142780">{{cite journal |vauthors=Zhang S |title=Natural history of mevalonate kinase deficiency: a literature review |journal=Pediatr Rheumatol Online J |volume=14 |issue=1 |pages=30 |date=May 2016 |pmid=27142780 |pmc=4855321 |doi=10.1186/s12969-016-0091-7 |url=}}</ref>
 
{| class="wikitable"
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Category of Disease
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Diseases
! colspan="14" align="center" style="background:#4479BA; color: #FFFFFF;" + |Signs and symptoms
! colspan="5" align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory findings
|-
| rowspan="2" |'''Inheritance pattern'''
| rowspan="2" |'''Fever duration'''
| rowspan="2" |'''Frequency of attacks'''
| rowspan="2" |'''Abdominal pain'''
| rowspan="2" |'''Arthralgia/Arthritis'''
| rowspan="2" |'''Chest pain'''
| rowspan="2" |'''Skin rash'''
| rowspan="2" |'''Myalgia/Body pain'''
| rowspan="2" |'''Diarrhea/Vomiting'''
| rowspan="2" |'''Neurologic manifestations'''
| rowspan="2" |'''Conjunctivitis'''
| rowspan="2" |'''Aphthous stomatitis'''
| rowspan="2" |'''Lymphadenopathy'''
| rowspan="2" |'''Splenomegaly'''
| rowspan="2" |'''Complete blood count (CBC)'''
| rowspan="2" |'''C- reactive protein (CRP)'''
|-
|'''Erythrocyte sedimentation rate (ESR)'''
|'''Other findings'''
|'''Genetic analysis'''
|-
| rowspan="11" |
===Autoinflammatory diseases===
| '''[[Familial mediterranean fever]]'''<ref>{{Cite journal
| author = [[M. Medlej-Hashim]], [[I. Petit]], [[S. Adib]], [[E. Chouery]], [[N. Salem]], [[V. Delague]], [[M. Rawashdeh]], [[I. Mansour]], [[G. Lefranc]], [[R. Naman]], [[J. Loiselet]], [[J. C. Lecron]], [[J. L. Serre]] & [[A. Megarbane]]
  | title = Familial Mediterranean Fever: association of elevated IgD plasma levels with specific MEFV mutations
| journal = [[European journal of human genetics : EJHG]]
| volume = 9
| issue = 11
| pages = 849–854
| year = 2001
| month = November
| doi = 10.1038/sj.ejhg.5200725
| pmid = 11781702
}}</ref><ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref>
|
* [[Autosomal recessive]]/[[Autosomal Dominant|dominant]]
|
* <nowiki>12-72 h</nowiki>
|
* <nowiki>Weekly or 3-4 times/year</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
*[[erysipelas]]-like [[erythema]]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
*[[Headache]]
|<nowiki>-/+</nowiki>
|<nowiki>-/+</nowiki>
|<nowiki>-/+</nowiki>
|<nowiki>+</nowiki>
|
* [[Leukocytosis]]
|'''↑'''
|'''↑'''
|
* High risk for [[amyloidosis]]
|
* [[Mutation]] in [[MEFV]] [[gene]]
|-
| '''[[Hyperimmunoglobulinemia D with recurrent fever|Hyper IgD with recurrent fever]]'''<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref><ref name="KrausCulican2009">{{cite journal|last1=Kraus|first1=Courtney L|last2=Culican|first2=Susan M|title=Nummular keratopathy in a patient with Hyper-IgD Syndrome|journal=Pediatric Rheumatology|volume=7|issue=1|year=2009|issn=1546-0096|doi=10.1186/1546-0096-7-14}}</ref><ref name="Mulders-MandersSimon2015">{{cite journal|last1=Mulders-Manders|first1=C. M.|last2=Simon|first2=A.|title=Hyper-IgD syndrome/mevalonate kinase deficiency: what is new?|journal=Seminars in Immunopathology|volume=37|issue=4|year=2015|pages=371–376|issn=1863-2297|doi=10.1007/s00281-015-0492-6}}</ref>
|
* [[Autosomal recessive]]
|
* <nowiki>3-7 days</nowiki>
|
* <nowiki>Every 2-12 weeks</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
* Diffuse [[maculopapular rash]]
* Polymorphous [[rash]]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
*[[Headache]]
| -
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|
*[[Leukocytosis]]
|'''↑'''
|'''↑'''
|
* Elevated srum level of [[IgD]] & [[IgA]]
* Chronic [[mevalonic aciduria]]
|
* [[Mutation]] in [[Mevalonate kinase|MVK]] [[gene]]
|-
| '''[[TNF receptor associated periodic syndrome|TNF receptor-associated periodic syndrome]]'''<ref name="ToroAksentijevich2000">{{cite journal|last1=Toro|first1=Jorge R.|last2=Aksentijevich|first2=Ivona|last3=Hull|first3=Keith|last4=Dean|first4=Jane|last5=Kastner|first5=Daniel L.|title=Tumor Necrosis Factor Receptor–Associated Periodic Syndrome|journal=Archives of Dermatology|volume=136|issue=12|year=2000|issn=0003-987X|doi=10.1001/archderm.136.12.1487}}</ref><ref name="LachmannPapa2014">{{cite journal|last1=Lachmann|first1=H J|last2=Papa|first2=R|last3=Gerhold|first3=K|last4=Obici|first4=L|last5=Touitou|first5=I|last6=Cantarini|first6=L|last7=Frenkel|first7=J|last8=Anton|first8=J|last9=Kone-Paut|first9=I|last10=Cattalini|first10=M|last11=Bader-Meunier|first11=B|last12=Insalaco|first12=A|last13=Hentgen|first13=V|last14=Merino|first14=R|last15=Modesto|first15=C|last16=Toplak|first16=N|last17=Berendes|first17=R|last18=Ozen|first18=S|last19=Cimaz|first19=R|last20=Jansson|first20=A|last21=Brogan|first21=P A|last22=Hawkins|first22=P N|last23=Ruperto|first23=N|last24=Martini|first24=A|last25=Woo|first25=P|last26=Gattorno|first26=M|title=The phenotype of TNF receptor-associated autoinflammatory syndrome (TRAPS) at presentation: a series of 158 cases from the Eurofever/EUROTRAPS international registry|journal=Annals of the Rheumatic Diseases|volume=73|issue=12|year=2014|pages=2160–2167|issn=0003-4967|doi=10.1136/annrheumdis-2013-204184}}</ref>
|
* [[Autosomal dominant]]
|
* <nowiki>3-4 weeks</nowiki>
|
* <nowiki>Variable</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|
* Migrating [[rash]] with deep [[pain]] under the areas with the [[rash]]
* Severe [[pain]] follows the [[rash]] path in a centrifugal pattern
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
*[[Headache]]
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|
*[[Leukocytosis]]
|'''↑'''
|'''↑'''
|
* [[Periorbital edema]]
|
* [[Mutation]] in TNFRSF1A [[gene]]
|-
|'''[[Muckle-Wells syndrome|Muckle-Wells Syndrome]]'''<ref name="HawkinsLachmann2004">{{cite journal|last1=Hawkins|first1=Philip N.|last2=Lachmann|first2=Helen J.|last3=Aganna|first3=Ebun|last4=McDermott|first4=Michael F.|title=Spectrum of clinical features in Muckle-Wells syndrome and response to anakinra|journal=Arthritis & Rheumatism|volume=50|issue=2|year=2004|pages=607–612|issn=0004-3591|doi=10.1002/art.20033}}</ref><ref name="AhmadiBrewer2011">{{cite journal|last1=Ahmadi|first1=Neda|last2=Brewer|first2=Carmen C.|last3=Zalewski|first3=Christopher|last4=King|first4=Kelly A.|last5=Butman|first5=John A.|last6=Plass|first6=Nicole|last7=Henderson|first7=Cailin|last8=Goldbach-Mansky|first8=Raphaela|last9=Kim|first9=H. Jeffrey|title=Cryopyrin-Associated Periodic Syndromes|journal=Otolaryngology–Head and Neck Surgery|volume=145|issue=2|year=2011|pages=295–302|issn=0194-5998|doi=10.1177/0194599811402296}}</ref>
|
* [[Autosomal dominant]]
|
* 2-3 days
|
* <nowiki>More common during cold seasons</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|
*[[Urticaria]]-like [[rash]]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
*[[Headache]]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| -
| -
|
* [[Leukocytosis]]
|'''↑'''
|'''↑'''
|
* Cold-triggered attacks
|
* [[Mutation]] in NLRP3 [[gene]]
|-
|[[Familial cold urticaria|'''Familial cold urticaria''']]<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref><ref name="StychDobrovolny2008">{{cite journal|last1=Stych|first1=Beate|last2=Dobrovolny|first2=Diana|title=Familial cold auto-inflammatory syndrome (FCAS): characterization of symptomatology and impact on patients' lives|journal=Current Medical Research and Opinion|volume=24|issue=6|year=2008|pages=1577–1582|issn=0300-7995|doi=10.1185/03007990802081543}}</ref>
|
* [[Autosomal dominant]]
|
* 12-24 hours, or longer
|
* Common in cold seasons
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|
* [[Urticaria]]-like [[rash]]
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
* [[Headache]]
* Mild [[hearing loss]]
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
* [[Leukocytosis]]
|'''↑'''
|'''↑'''
|
* Cold-triggered attacks
|
* [[Mutation]] in NLRP3 [[gene]]
|-
|[[Neonatal onset multisystem inflammatory disease|'''Neonatal onset multisystem inflammatory disease''']]<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref><ref name="Goldbach-ManskyDailey2006">{{cite journal|last1=Goldbach-Mansky|first1=Raphaela|last2=Dailey|first2=Natalie J.|last3=Canna|first3=Scott W.|last4=Gelabert|first4=Ana|last5=Jones|first5=Janet|last6=Rubin|first6=Benjamin I.|last7=Kim|first7=H. Jeffrey|last8=Brewer|first8=Carmen|last9=Zalewski|first9=Christopher|last10=Wiggs|first10=Edythe|last11=Hill|first11=Suvimol|last12=Turner|first12=Maria L.|last13=Karp|first13=Barbara I.|last14=Aksentijevich|first14=Ivona|last15=Pucino|first15=Frank|last16=Penzak|first16=Scott R.|last17=Haverkamp|first17=Margje H.|last18=Stein|first18=Leonard|last19=Adams|first19=Barbara S.|last20=Moore|first20=Terry L.|last21=Fuhlbrigge|first21=Robert C.|last22=Shaham|first22=Bracha|last23=Jarvis|first23=James N.|last24=O'Neil|first24=Kathleen|last25=Vehe|first25=Richard K.|last26=Beitz|first26=Laurie O.|last27=Gardner|first27=Gregory|last28=Hannan|first28=William P.|last29=Warren|first29=Robert W.|last30=Horn|first30=William|last31=Cole|first31=Joe L.|last32=Paul|first32=Scott M.|last33=Hawkins|first33=Philip N.|last34=Pham|first34=Tuyet Hang|last35=Snyder|first35=Christopher|last36=Wesley|first36=Robert A.|last37=Hoffmann|first37=Steven C.|last38=Holland|first38=Steven M.|last39=Butman|first39=John A.|last40=Kastner|first40=Daniel L.|title=Neonatal-Onset Multisystem Inflammatory Disease Responsive to Interleukin-1β Inhibition|journal=New England Journal of Medicine|volume=355|issue=6|year=2006|pages=581–592|issn=0028-4793|doi=10.1056/NEJMoa055137}}</ref><ref name="KimMontealegre Sanchez2014">{{cite journal|last1=Kim|first1=Hanna|last2=Montealegre Sanchez|first2=Gina A.|last3=Chapelle|first3=Dawn C.|last4=Plass|first4=Nicole|last5=Dwyer|first5=Andrew|last6=Goldbach-Mansky|first6=Raphaela|last7=Hill|first7=Suvimol|title=A80: Skeletal Features of Neonatal-Onset Multisystem Inflammatory Disease (NOMID) on Anakinra Treatment: Long-Term Follow-up|journal=Arthritis & Rheumatology|volume=66|year=2014|pages=S113–S113|issn=23265191|doi=10.1002/art.38496}}</ref>
|
* [[Autosomal dominant]]
|
* Continuous
|
* Common in cold seasons
|<nowiki>+</nowiki>
|
|<nowiki>+</nowiki>
|
* [[Urticaria]]-like [[rash]]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
* [[Headache]]
* [[Aseptic meningitis]]
* [[Papilledema]]
* [[Seizure]]
* [[sensorineural hearing loss]]
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|
* [[Generalized lymphadenopathy]]
|<nowiki>+</nowiki>
|
* [[Leukocytosis]]
|'''↑'''
|'''↑'''
|
* [[Intellectual disability]]
* Elevated levels of [[Immunoglobulins]]
* Cold-triggered attacks
|
* [[Mutation]] in NLRP3 [[gene]]
|-
|'''[[Pyogenic sterile arthritis, pyoderma gangrenosum, acne]] ([[Papa syndrome]])'''<ref name="YeonLindor2000">{{cite journal|last1=Yeon|first1=Howard B.|last2=Lindor|first2=Noralane M.|last3=Seidman|first3=J.G.|last4=Seidman|first4=Christine E.|title=Pyogenic Arthritis, Pyoderma Gangrenosum, and Acne Syndrome Maps to Chromosome 15q|journal=The American Journal of Human Genetics|volume=66|issue=4|year=2000|pages=1443–1448|issn=00029297|doi=10.1086/302866}}</ref><ref name="SchellevisStoffels2011">{{cite journal|last1=Schellevis|first1=M. A.|last2=Stoffels|first2=M.|last3=Hoppenreijs|first3=E. P. A. H.|last4=Bodar|first4=E.|last5=Simon|first5=A.|last6=van der Meer|first6=J. W. M.|title=Variable expression and treatment of PAPA syndrome|journal=Annals of the Rheumatic Diseases|volume=70|issue=6|year=2011|pages=1168–1170|issn=0003-4967|doi=10.1136/ard.2009.126185}}</ref>
|
* [[Autosomal dominant]]
|
* <nowiki>Variable</nowiki>
|
* <nowiki>Variable</nowiki>
|<nowiki>+/-</nowiki>
|
* Destructive [[arthritis]]
|<nowiki>+/-</nowiki>
|
*[[Pyoderma gangrenosum]] [[Ulceration|ulcerative]] [[Lesion|lesions]]
* Severe [[cystic acne]]
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
*[[Leukocytosis]]
|'''↑'''
|'''↑'''
|
* High [[Neutrophil|neutrophilic]] content of [[synovial fluid]]
* Negative [[Culture collection|cultures]] of [[bone]] and [[skin]]
|
* [[Mutation]] in PSTPIP1 [[gene]]
|-
| '''[[Periodic fever, aphthous stomatitis, pharyngitis and adenitis|Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA)]]'''<ref name="VanoniFederici2018">{{cite journal|last1=Vanoni|first1=Federica|last2=Federici|first2=Silvia|last3=Antón|first3=Jordi|last4=Barron|first4=Karyl S.|last5=Brogan|first5=Paul|last6=De Benedetti|first6=Fabrizio|last7=Dedeoglu|first7=Fatma|last8=Demirkaya|first8=Erkan|last9=Hentgen|first9=Veronique|last10=Kallinich|first10=Tilmann|last11=Laxer|first11=Ronald|last12=Russo|first12=Ricardo|last13=Toplak|first13=Natasa|last14=Uziel|first14=Yosef|last15=Martini|first15=Alberto|last16=Ruperto|first16=Nicolino|last17=Gattorno|first17=Marco|last18=Hofer|first18=Michael|title=An international delphi survey for the definition of the variables for the development of new classification criteria for periodic fever aphtous stomatitis pharingitis cervical adenitis (PFAPA)|journal=Pediatric Rheumatology|volume=16|issue=1|year=2018|issn=1546-0096|doi=10.1186/s12969-018-0246-9}}</ref><ref name="CattaliniSoliani2015">{{cite journal|last1=Cattalini|first1=Marco|last2=Soliani|first2=Martina|last3=Rigante|first3=Donato|last4=Lopalco|first4=Giuseppe|last5=Iannone|first5=Florenzo|last6=Galeazzi|first6=Mauro|last7=Cantarini|first7=Luca|title=Basic Characteristics of Adults with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenopathy Syndrome in Comparison with the Typical Pediatric Expression of Disease|journal=Mediators of Inflammation|volume=2015|year=2015|pages=1–11|issn=0962-9351|doi=10.1155/2015/570418}}</ref><ref name="GattornoCaorsi2009">{{cite journal|last1=Gattorno|first1=M.|last2=Caorsi|first2=R.|last3=Meini|first3=A.|last4=Cattalini|first4=M.|last5=Federici|first5=S.|last6=Zulian|first6=F.|last7=Cortis|first7=E.|last8=Calcagno|first8=G.|last9=Tommasini|first9=A.|last10=Consolini|first10=R.|last11=Simonini|first11=G.|last12=Pelagatti|first12=M. A.|last13=Baldi|first13=M.|last14=Ceccherini|first14=I.|last15=Plebani|first15=A.|last16=Frenkel|first16=J.|last17=Sormani|first17=M. P.|last18=Martini|first18=A.|title=Differentiating PFAPA Syndrome From Monogenic Periodic Fevers|journal=PEDIATRICS|volume=124|issue=4|year=2009|pages=e721–e728|issn=0031-4005|doi=10.1542/peds.2009-0088}}</ref>
|
* <nowiki>Unkown</nowiki>
|
* <nowiki>3-6 days</nowiki>
|
* <nowiki>Every 21-28 days</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* Cervical [[lymphadenopathy]]
|<nowiki>-</nowiki>
|
*[[Leukocytosis]]
|'''↑'''
|'''↑'''
|
* Culture negative [[exudative]] [[pharyngitis]] is a classic finding
|
* Unknown
|-
|'''[[Blau syndrome]]'''<ref name="RoséAróstegui2009">{{cite journal|last1=Rosé|first1=Carlos D.|last2=Aróstegui|first2=Juan I.|last3=Martin|first3=Tammy M.|last4=Espada|first4=Graciela|last5=Scalzi|first5=Lisabeth|last6=Yagüe|first6=Jordi|last7=Rosenbaum|first7=James T.|last8=Modesto|first8=Consuelo|last9=Cristina Arnal|first9=Maria|last10=Merino|first10=Rosa|last11=García-Consuegra|first11=Julia|last12=Carballo Silva|first12=María Antonia|last13=Wouters|first13=Carine H.|title=NOD2-Associated pediatric granulomatous arthritis, an expanding phenotype: Study of an international registry and a national cohort in spain|journal=Arthritis & Rheumatism|volume=60|issue=6|year=2009|pages=1797–1803|issn=00043591|doi=10.1002/art.24533}}</ref><ref name="KimPark2016">{{cite journal|last1=Kim|first1=Woojoong|last2=Park|first2=Eujin|last3=Ahn|first3=Yo Han|last4=Lee|first4=Jiwon M.|last5=Kang|first5=Hee Gyung|last6=Kim|first6=Byung Joo|last7=Ha|first7=Il-Soo|last8=Cheong|first8=Hae Il|title=A familial case of Blau syndrome caused by a novelNOD2genetic mutation|journal=Korean Journal of Pediatrics|volume=59|issue=Suppl 1|year=2016|pages=S5|issn=1738-1061|doi=10.3345/kjp.2016.59.11.S5}}</ref>
|
* [[Autosomal dominant]]
|
* <nowiki>Intermittent or persistent daily fever</nowiki>
|
* Variable
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|
* scaly [[plaques]]
* Non-caseating [[granulomatous]] [[dermatitis]] in [[Biopsy]]
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|
*[[Cranial]] [[neuropathies]]
*[[Visual]] problems in 80%
*[[Peripheral neuropathy|Peripheral neuropathies]]
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|
* [[Leukocytosis]]
|'''↑'''
|'''↑'''
|
* [[Ophthalmologic]] manifestations are common
|
* [[Mutation]] in NOD2 [[gene]]
|}
 
{| class="wikitable"
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Category of Disease
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Diseases
! colspan="10" align="center" style="background:#4479BA; color: #FFFFFF;" + |Signs and symptoms
! colspan="11" align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory findings
|-
| rowspan="2" |'''Fever'''
| rowspan="2" |'''Fatigue'''
| rowspan="2" |'''Arthralgia'''
| rowspan="2" |'''Myalgia'''
| rowspan="2" |'''Soft tissue swelling/serositis'''
| rowspan="2" |'''Skin rash'''
| rowspan="2" |'''Weight loss'''
| rowspan="2" |'''Dyspnea'''
| rowspan="2" |'''Sore throat'''
| rowspan="2" |'''Lymphadenopathy'''
| rowspan="2" |'''Complete blood count (CBC)'''
| rowspan="2" |'''Liver function tests (LFTs)'''
| colspan="2" |
=== Inflammatory markers ===
| colspan="6" |
=== Autoantibodies ===
|
=== Diagnostic tests ===
|-
|'''Erythrocyte sedimentation rate (ESR)'''
|'''C- reactive protein (CRP)'''
|'''Anti-nuclear antibodies (ANA)'''
|'''Rheumatoid factor (RF)'''
|'''Anti- glomerular basement membrane (anti-GBM)'''
|'''Anti-dsDNA'''
|'''Anti-Jo1/ Anti Mi2'''
|'''ANCA'''
|
|-
| rowspan="9" |
=== Infections ===
| [[Human Immunodeficiency Virus (HIV)|'''HIV''']]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+ /-</nowiki>
|<nowiki>+</nowiki>
|
* [[Leukopenia]]
* [[Anemia]]
* [[Thrombocytopenia]]
|
* [[Alanine transaminase|ALT]]'''↑'''
* [[Aspartate transaminase|AST]]'''↑'''
|'''↑'''
|'''↑'''
| -
| -
| -
| -
| -
| -
|
|-
| ''[[Herpesviridae|'''Herpesviridae''']]''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
* Papular or vesicular
* [[Erythematous]] base
* Painful
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|
* [[Leukocytosis]]
|<nowiki>-</nowiki>
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| -
| -
|
|-
| '''[[Measles]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|
* 3-5 days after fever
* [[Erythematous]] [[maculopapular]] rash starts on face spreads to trunk and [[extremities]] (centrifugal)
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
* [[Leukocytosis]]
|<nowiki>-</nowiki>
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| -
| -
|
|-
| '''[[Hepatitis|Viral hepatitis]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|
* [[Leukocytosis]]
|
* [[Alanine transaminase|ALT]]'''↑'''
* [[Aspartate transaminase|AST]]'''↑'''
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| -
| -
|
|-
| ''[[Parvovirus B19|'''Parvovirus B19''']]''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
|
* Slapped cheek rash
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* [[Leukopenia]]
* [[Aplastic anemia]] (low [[reticulocyte]] count)
* [[Thrombocytopenia]]
|
* [[Alanine transaminase|ALT]]'''↑'''
* [[Aspartate transaminase|AST]]'''↑'''
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| -
| -
|
|-
|'''[[Infective endocarditis]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
|
* [[Purpuric]]
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* [[Leukocytosis]]
|<nowiki>-</nowiki>
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| -
| -
|Blood cultures, ultrasonography
|-
|'''[[Borreliosis]], [[Brucellosis]], [[Yersiniosis]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|
* Target rash ([[Borrelia]])
* Diffuse [[maculopapular]] ([[Brucellosis]])
* [[Erythema nodosum]] ([[Yersiniosis]])
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* [[Leukocytosis]]
|
* [[Alanine transaminase|ALT]]'''↑'''
* [[Aspartate transaminase|AST]]'''↑'''
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| -
| -
|Serology, PCR
|-
|'''[[Syphilis]] and [[Jarisch-Herxheimer reaction]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|
* Non-pruritic
* [[Macular]]
* On palms and soles
* Round
* Seen in [[secondary syphilis]]
* [[Penis|Penile]] [[ulceration]] (painless)
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
* [[Leukocytosis]]
|
* [[Alanine transaminase|ALT]]'''↑''' (Uncommon)
* [[Aspartate transaminase|AST]]'''↑''' (Uncommon)
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| -
| -
|Serology, PCR
|-
|'''[[Toxoplasmosis]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|
* [[Maculopapular]]
* Palms and soles
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
* [[Leukocytosis]] (eosinophilia)
|
|
|
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| -
| -
|Serology, PCR
|-
|
| colspan="22" |
|-
| rowspan="3" |
=== Neoplasia ===
|'''[[Malignant lymphoma]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|
* Purplish scaly rash in cases of [[cutaneous]] [[lymphoma]]
|<nowiki>+</nowiki>
|<nowiki>+ </nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* [[Anemia of chronic disease]]
|
* In case of [[liver]] [[metastasis]] ([[Non-Hodgkin lymphoma]]):
** [[Alanine transaminase|ALT]]'''↑'''
** [[Aspartate transaminase|AST]]'''↑'''
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| -
| -
|CT, PET/CT, Bone marrow examination, lymph node biopsy
|-
|'''[[Castleman's disease|Multicentric Castleman disease]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* [[Anemia]]
* [[Thrombocytopenia]]
|<nowiki>-</nowiki>
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| -
| -
|Lymph node biopsy
|-
|'''[[Angioimmunoblastic T-cell lymphoma|Angioimmunoblastic T cell lymphoma]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
* [[Maculopapular]] eruption on the trunk mimicking [[Erythema toxicum|toxic erythema]]
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* [[Lymphocytosis]]
|
* [[Alanine transaminase|ALT]]'''↑'''
* [[Aspartate transaminase|AST]]'''↑'''
|'''↑'''
|↑
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| -
| -
|Lymph node biopsy
|-
|
| colspan="22" |
|-
|
=== Drug hypersensitivity ===
|Drug reaction with eosinophilia and systemic symptoms
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|
* [[Urticaria|Utricaria]] (Immediate type)
* [[Maculopapular]] (Delayed type)
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
* [[Leukocytosis]] ([[eosinophilia]])
|<nowiki>-</nowiki>
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| -
| -
|Eosinophil count, skin biopsy
|-
|
| colspan="22" |
|-
| rowspan="7" |Autoimmune conditions
|'''[[Systemic lupus erythematosus]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|
* Butter-fly shaped [[erythematous]] malar rash that spares the [[nasolabial folds]]
* Coin-shaped [[erythematous]] rash on extremities or trunk
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|
* [[Leukopenia]]
* [[Hemolytic anemia]]
* [[Thrombocytopenia]]
|
* [[ALT]]'''↑'''
* [[AST]]'''↑'''
|'''↑'''
|'''↑'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
| -
| -
|Antinuclear autoantibodies
|-
|'''Inflammatory myositis'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+ (weakness > pain)</nowiki>
|<nowiki>-</nowiki>
|
* [[Macular]] red rash over the back of the fingers, elbows or knees (Grotton's sign)
* [[Macular]] purpish or reddish rash on the upper chest or back  (Shawl-like, heliotrope rash)
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|
* [[Leukocytosis]]
|<nowiki>-</nowiki>
|'''↑'''
|'''↑'''
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| +
| -
|Idem, muscle biopsy
|-
|[[Rheumatoid arthritis|'''Rheumatoid arthritis''']]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* [[Purpura]]
* [[Ulcers]]
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* [[Anemia of chronic disease]]
* [[Neutropenia]] ([[Felty's syndrome]])
|<nowiki>-</nowiki>
|'''↑'''
|'''↑'''
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| -
| -
|Anti-citrullinated peptids autoantibodies, rheumatoid factor
|-
|'''[[Systemic vasculitides]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* Nasopharyngeal ulceration ([[Wegener's granulomatosis|Wegner's granulomatosis]])
* [[Erythematous]] rash on palms and soles ([[Kawasaki disease]])
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|
* [[Leukocytosis]]
|<nowiki>-</nowiki>
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
| -
| +
|ANCA, tissue biopsy, arteriography
|-
|'''[[Familial mediterranean fever|Familial Mediterranean fever]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
* Red rash on [[lower extremities]] (ankles and knees)
|<nowiki>+</nowiki>
|<nowiki>+ (due to pain)</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|
* [[Leukocytosis]] (during acute flares)
|<nowiki>-</nowiki>
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|Familial history, MEFV gene analysis
|-
|'''[[Mevalonate kinase deficiency]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|
* [[Maculopapular]] rash
* [[Aphthous ulcers]] (or [[stomatitis]]) 
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
* [[Leukocytosis]] (during acute flares)
|<nowiki>-</nowiki>
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|Urinary mevalonic acid, mevalonate kinase analysis
|-
|'''[[Reactive arthritis]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
* [[Keratoderma blennorrhagica]] (palms, soles, scrotum)
* Circinate balanitis (penile)
|<nowiki>-</nowiki>
| + ([[Aortic insufficiency]])
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* [[Leukocytosis]]
|<nowiki>-</nowiki>
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|HLA B27, magnetic resonance imaging
|-
|
| colspan="22" |
|-
|
=== Miscellaneous ===
|'''[[Sarcoidosis]]'''
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* Waxy skin [[plaques]]
* Violaceous facial lesions
* [[Erythema nodosum]]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* [[Lymphopenia]]
* [[Eosinophilia]]
|
* Normal [[Alanine transaminase|ALT]], [[Aspartate transaminase|AST]]
* [[Alkaline phosphatase|ALP]] '''↑ (infiltrative pattern)'''
|'''↑'''
|'''↑'''
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
* Lymph node/Lung biopsy
* ACE levels
* FDG-PET
|}
 
==Less common differentials==
 
FMF must be differentiated from other causes of secondary peritonitis
 
{| style="margin: 1em 1em 1em 0; background: #f9f9f9; border: 1px #aaa solid; border-collapse: collapse;" cellspacing="0" cellpadding="4" border="2"
|+'''Differentiating FMF from other causes of peritonitis'''
! colspan="2" style="background: #4479BA; text-align: center;" | {{fontcolor|#FFF| '''Disease'''}}
! colspan="1" style="background: #4479BA; text-align: center;" | {{fontcolor|#FFF| '''Prominent clinical findings'''}}
! colspan="1" style="background: #4479BA; text-align: center;" | {{fontcolor|#FFF| '''Lab tests'''}}
! colspan="1" style="background: #4479BA; text-align: center;" | {{fontcolor|#FFF| '''Tratment'''}}
|-
| rowspan="3" |'''Primary peritonitis'''
|'''[[Primary peritonitis|Spontaneous bacterial peritonitis]]'''
|
* Absence of GI [[perforation]], most closely associated with [[cirrhosis]] and [[Liver disease|advanced liver disease]].
* Presents with abrupt onset of [[fever]], [[abdominal pain]], [[distension]], and [[rebound tenderness]].
|
* Most have clinical and biochemical manifestations of advanced [[cirrhosis]] or [[nephrosis]] like [[leukocytosis]],[[hypoalbuminemia]],
* Prolonged [[prothrombin]] time. SAAG >1.1 g/dL, increased serum [[lactic acid]] level, or a decreased [[Ascites|ascitic fluid]] pH (< 7.31) supports the diagnosis. [[Gram staining]] reveals bacteria in only 25% of cases.
* Diagnosed by analysis of the [[Ascitic|ascitic fluid]] which reveals [[WBC]] > 500/ML, and [[PMN]] >250cells/ml.
* [[Culture medium|Culture]] of ascitic fluid inoculated immediately into [[blood culture]] media at the bedside usually reveals a single [[Enteric Bacilli|enteric organism]], most commonly ''[[Escherichia coli]]'', ''[[Klebsiella]]'', or [[streptococci]].
|
* Once diagnosed,it is treated with [[Ceftriaxone]].
|-
|'''[[Tuberculous peritonitis]]'''
|
* Seen in 0.5% of new cases of [[tuberculosis]] particularly in young women in endemic areas as a primary infection.
* Presents with [[abdominal pain]] and [[distension]], [[fever]], [[night sweats]], [[weight loss]], and altered bowel habits.
|
* [[Ascites]] is present in about half of cases. [[Abdominal mass]] may be felt in a third of cases. The [[peritoneal fluid]] is characterized by a [[protein]] concentration > 3 g/dL with < 1.1 g/dL SAAG and [[Lymphocyte|lymphocyte predominance]] of [[WBC]].
* Definitive diagnosis in 80% of cases is by culture. Most patients presenting acutely are diagnosed only by [[laparotomy]].
|
* Combination [[Antituberculosis|antituberculosis chemotherapy]] is preferred in chronic cases.
|-
|'''[[Continuous ambulatory peritoneal dialysis|Continuous Ambulatory Peritoneal Dialysis]]''' [[Continuous ambulatory peritoneal dialysis|('''CAPD peritonitis)''']]
|
* [[Peritonitis]] is one of the major complications of [[peritoneal dialysis]] & 72.6% occurred within the first six months of [[peritoneal dialysis]].
* Historically, [[coagulase-negative staphylococci]] were the most common cause of peritonitis in [[Continuous ambulatory peritoneal dialysis|CAPD]], presumably due to touch contamination or infection via the pericatheter route.
* Treatment for [[peritoneal dialysis]]-associated peritonitis consists of [[Antimicrobial drug|antimicrobial therapy]], in some cases catheter removal is also warranted.
* Additional therapies for [[Peritonitis|relapsing or recurrent peritonitis]] may include [[Fibrinolytic agent|fibrinolytic agents]] and [[peritoneal lavage]]. Most episodes of peritoneal dialysis-associated peritonitis resolve with outpatient [[Antibiotic|antibiotic treatment]].
|
* Majority of [[peritonitis]] cases are caused by [[bacteria]] (50%-due to [[Gram-positive bacteria|gram positive]] organisms, 15% to [[gram negative]] organisms,20% were culture negative.2% of cases are caused by [[fungi]], mostly [[Candida]] species. Polymicrobial infection in 4%.Exit-site infection was present in 13% and a [[peritoneal fluid]] leak in 3 % and [[M.tuberculosis]] 0.1%.
|
* [[Antibiotic|Initial empiric antibiotic coverage]] for peritoneal dialysis-associated peritonitis consists of coverage for [[gram-positive]] organisms (by [[vancomycin]] or a [[Cephalosporins|first-generation cephalosporin]]) and [[gram-negative]] organisms (by a [[cephalosporin|third-generation cephalosporin]] or an [[aminoglycoside]]). Subsequently, the regimen should be adjusted based on [[Culture medium|culture]] and [[sensitivity]] data. Cure rates are approximately 75%.
|-
| rowspan="2" |'''[[Secondary peritonitis]]'''
|'''Acute [[bacterial]] [[secondary peritonitis]]'''
|
* Occurs after perforating, penetrating, inflammatory, infectious, or [[ischemic]] injuries of the GI or GU tracts. Most often follows disruption of a hollow viscus?chemical peritonitis?bacterial peritonitis(polymicrobial, includes [[aerobic]] [[gram negative]] {[[E coli]], [[Klebsiella]], [[Enterobacter]], [[Proteus mirabilis]]} and gram positive { [[Enterococcus]], [[Streptococcus]]} and [[anaerobes]] {[[Bacteroides]], [[clostridia]]}).
* Presents with [[abdominal pain]], [[tenderness]], [[guarding]] or rigidity, [[distension]], free peritoneal air, and diminished [[bowel sounds]]. Signs that reflect irritation of the parietal peritoneum resulting [[ileus]]. Systemic findings include [[fever]], [[chills]] or [[rigors]], [[tachycardia]], [[sweating]], [[tachypnea]], [[restlessness]], [[dehydration]], [[oliguria]], [[disorientation]], and, ultimately, refractory [[shock]].
|
|
* [[Peritoneal lavage]], [[Laparoscopy]] are the treatment of choice.
|-
|'''[[Biliary]] [[Secondary peritonitis|peritonitis]]'''
|
* Most often seen in cases of rupture of pathological [[gallbladder]] or [[bile duct]] or [[Cholangitis|cholangitic abscess]] or secondary to obstruction of  the [[biliary tract]].
* Seen in alcoholic patients with [[ascites]].
|
|
|-
| colspan="2" |'''[[Peritonitis|Tertiary peritonitis]]'''
|
* Persistence or recurrence of [[Infection|intraabdominal infection]] following apparently adequate therapy of [[Peritonitis|primary or secondary peritonitis]].
* Associated with [[Mortality|high mortality]] due to multi organ dysfunction. It presents in a similar way as other [[peritonitis]] but is recognized as an adverse outcome with poor prognosis.
|
* [[Enterococcus]], [[Candida]], [[Staphylococcus epidermidis]], and [[Enterobacter]] being the most common organisms.
|
* Characterized by lack of response to appropriate surgical and [[antibiotic therapy]] due to disturbance in the hosts [[immune response]].
|-
| colspan="2" |'''[[Familial mediterranean fever|Familial Mediterranean fever (periodic peritonitis, familial paroxysmal polyserositis)]]'''
|
* Rare [[Genetic disorder|genetic condition]] which affects individuals of Mediterranean genetic background.
* Etiology is unclear.
* Presents with recurrent bouts of [[abdominal pain]] and [[tenderness]] along with [[pleuritic]] or [[joint pain]]. [[Fever]] and [[leukocytosis]] are common.
|
|
* [[Colchicine]] prevents but does not treat acute attacks.
|-
| colspan="2" |'''[[Granulomatous peritonitis]]'''
|
* A rare condition caused by disposable surgical fabrics or food particles from a [[perforated ulcer]], eliciting a vigorous [[granulomatous]] ([[Hypersensitivity|delayed hypersensitivity]]) response in some patients 2-6 weeks after [[laparotomy]].
* Presents with [[abdominal pain]], [[fever]], [[nausea and vomiting]], [[ileus]], and systemic complaints, mild and diffuse [[abdominal tenderness]].
|
* Diagnosed by the demonstration of diagnostic Maltese cross pattern of starch particles.
|
* The disease is self-limiting.
* Treated with [[corticosteroids]] or [[Anti inflammatory medications|anti-inflammatory agents]].
|-
| colspan="2" |'''[[Sclerosing encapsulating peritonitis]]'''
|
* Seen in conditions associated with long term [[peritoneal dialysis]], shunts like [[Ventriculoperitoneal shunt|VP shunts]], history of [[Abdominal surgery|abdominal surgeries]], [[liver transplantation]].
* Symptoms include [[nausea]], [[abdominal pain]], [[diarrhea]], [[anorexia]], bloody [[ascites]].
|
|
|-
| colspan="2" |'''[[Abscess|Intraperitoneal abscesses]]'''
|
* Most common etiologies being [[Perforation|Gastrointestinal perforations]], postoperative complications, and penetrating injuries.
* Signs and symptoms depend on the location of the [[abscess]] within the [[peritoneal cavity]] and the extent of involvement of the surrounding structures.
* Diagnosis is suspected in any patient with a predisposing condition. In a third of cases it occurs as a sequela of [[Peritonitis|generalized peritonitis]].
* The pathogenic organisms are similar to those responsible for [[peritonitis]], but [[anaerobic]] organisms occupy an important role.
* The [[mortality rate]] of serious [[Abscesses|intra-abdominal abscesses]] is about 30%.
|
* Diagnosed best by [[CT-scans|CT]] scan of the abdomen.
|
* Treatment consists of prompt and complete [[CT]] or [[Ultrasound|US]] guided drainage of the [[abscess]], control of the primary cause, and adjunctive use of effective [[Antibiotics|antibiotics.]] Open drainage is reserved for [[abscesses]] for which percutaneous drainage is inappropriate or unsuccessful.
|-
| colspan="2" |'''[[Peritoneal mesothelioma]]'''
|
* Arises from the [[mesothelium]] lining the [[peritoneal cavity]].
* Its incidence is approximately 300-500 new cases being diagnosed in the United States each year. As with [[pleural mesothelioma]], there is an association with an [[Asbestos|asbestos exposure]].
* Most commonly affects men at the age of 50-69 years. Patients most often present with [[abdominal pain]] and later increased abdominal girth and [[ascites]] along with [[anorexia]], [[weight loss]] and [[abdominal pain]].
* Mean time from diagnosis to death is less than 1 year without treatment.  
|
* [[Computed tomography|CT]] with [[Contrast|intravenous contrast]] typically demonstrates the thickening of the [[peritoneum]]. [[Laparoscopy]] with tissue biopsy or CT guided tissue biopsy with [[immunohistochemical staining]] for [[calretinin]], [[cytokeratin|cytokeratin 5/6]], [[mesothelin]], and [[WT1|Wilms tumor 1 antigen]] remain the [[Gold standard (test)|gold standard]] for diagnosis.
|
* At [[laparotomy]] the goal is cytoreduction with [[excision]]. Debulking surgery and intraperitoneal [[chemotherapy]] improves survival in some cases.
|-
| colspan="2" |'''[[peritoneal carcinomatosis]]'''
|
* Associated with a history of [[ovarian]] or [[Malignancy|GI tract malignancy]].
* Symptoms include [[ascites]], [[abdominal pain]], [[nausea]], [[vomiting]].
|
|
|}
 
{| style="border: 0px; font-size: 90%; margin: 3px;" align=center
! colspan="2" rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" | Classification of acute abdomen based on etiology
! colspan="1" rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Presentation
! colspan="3" rowspan="1" align="center" style="background:#4479BA; color: #FFFFFF;" | Symptoms
! colspan="3" rowspan="1" align="center" style="background:#4479BA; color: #FFFFFF;" | Signs
! colspan="2" rowspan="1" align="center" style="background:#4479BA; color: #FFFFFF;" | Diagnosis
! colspan="1" rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" | Comments
|-
! colspan="1" rowspan="1" align="center" style="background:#4479BA; color: #FFFFFF;" | Fever
! align="center" style="background:#4479BA; color: #FFFFFF;" |Abdominal Pain
! align="center" style="background:#4479BA; color: #FFFFFF;" |Jaundice
! colspan="1" rowspan="1" align="center" style="background:#4479BA; color: #FFFFFF;" | Guarding
! align="center" style="background:#4479BA; color: #FFFFFF;" |Rebound Tenderness
! align="center" style="background:#4479BA; color: #FFFFFF;" |Bowel sounds
! colspan="1" rowspan="1" align="center" style="background:#4479BA; color: #FFFFFF;" | Lab Findings
! align="center" style="background:#4479BA; color: #FFFFFF;" |Imaging
|-
! colspan="1" rowspan="8" style="padding: 5px 5px; background: #DCDCDC;" align="center" | Common causes of Peritonitis
! colspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" | Primary Peritonitis
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" | [[Spontaneous bacterial peritonitis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Diffuse
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Hypoactive
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Ascitic fluid [[PMN]]>250 cells/mm<small>³</small>
 
* Culture: Positive for single organism
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Ultrasound for evaluation of liver cirrhosis
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|-
! colspan="1" rowspan="7" style="padding: 5px 5px; background: #DCDCDC;" align="center" | Secondary Peritonitis
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" | Perforated [[Gastric ulcer|gastric]] and [[duodenal ulcer]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | Diffuse
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |N
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Ascitic fluid
** [[LDH]] > serum [[LDH]]
 
** Glucose < 50mg/dl
 
** Total protein > 1g/dl
 
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Air under [[diaphragm]] in upright [[CXR]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Upper GI [[endoscopy]] for diagnosis
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" | Acute cholangitis
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | [[RUQ]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |N
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Abnormal [[LFT]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Ultrasound shows [[biliary]] dilatation
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Biliary drainage ([[Endoscopic retrograde cholangiopancreatography|ERCP]]) + IV antibiotics
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" | [[Acute cholecystitis|Acute cholecystitis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | [[RUQ]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Hypoactive
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[Hyperbilirubinemia]]
* [[Leukocytosis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Ultrasound shows gallstone and evidence of inflammation
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Murphy's sign|Murphy’s sign]]
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" | [[Acute pancreatitis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | [[Epigastric]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +/-
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |N
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Increased [[amylase]] / [[lipase]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Ultrasound shows evidence of [[inflammation]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Pain radiation to back
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" | [[Acute appendicitis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | RLQ
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Hypoactive
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Leukocytosis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Ultrasound shows evidence of [[inflammation]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Nausea and vomiting|Nausea & vomiting]], [[decreased appetite]]
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" | [[Diverticulitis|Acute diverticulitis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | LLQ
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +/-
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Hypoactive
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Leukocytosis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |CT scan and ultrasound shows evidence of inflammation
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" | [[Salpingitis|Acute salpingitis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | LLQ/ RLQ
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +/-
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +/-
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |N
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Leukocytosis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Pelvic ultrasound]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Vaginal discharge]]
|-
! colspan="2" rowspan="4" style="padding: 5px 5px; background: #DCDCDC;" align="center" | Hollow Viscous Obstruction
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" |Small intestine obstruction
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Diffuse
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | +/-
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Hyperactive then absent
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Leukocytosis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Abdominal X-ray|Abdominal X ray]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Nausea and vomiting|Nausea & vomiting]] associated with [[constipation]], [[Abdominal distension|abdominal distention]]
|-
|style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Volvulus]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Diffuse
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>+</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Hypoactive
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Leukocytosis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |CT scan and [[Abdominal x-ray|abdominal X ray]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Nausea and vomiting|Nausea & vomiting]] associated with [[constipation]], [[Abdominal distension|abdominal distention]]
|-
|style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Biliary colic]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |RUQ
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |+
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |N
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Increased [[bilirubin]] and [[alkaline phosphatase]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Ultrasound
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Nausea and vomiting|Nausea & vomiting]]
|-
|style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Renal colic]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Flank pain]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" | -
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |N
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Hematuria]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |CT scan and ultrasound
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Colicky [[abdominal pain]] associated with [[Nausea and vomiting|nausea & vomiting]]
|-
! rowspan="4" style="padding: 5px 5px; background: #DCDCDC;" align="center" |Vascular Disorders
! rowspan="2" style="padding: 5px 5px; background: #DCDCDC;" align="center" |Ischemic causes
|style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Mesenteric ischemia]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>+/-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Periumbilical
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Hyperactive
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Leukocytosis]] and [[lactic acidosis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |CT scan
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Nausea and vomiting|Nausea & vomiting]], normal physical examination
|-
|style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Ischemic colitis|Acute ischemic colitis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>+/-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Diffuse
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>+</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>+</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Hyperactive then absent
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Leukocytosis]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |CT scan
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Nausea and vomiting|Nausea & vomiting]]
|-
! rowspan="2" style="padding: 5px 5px; background: #DCDCDC;" align="center" |Hemorrhagic causes
|style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Ruptured abdominal aortic aneurysm]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Diffuse
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |N
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Normal
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |CT scan
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Unstable hemodynamics
|-
|style="padding: 5px 5px; background: #DCDCDC;" align="center" |Intra-abdominal or [[retroperitoneal hemorrhage]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Diffuse
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |N
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |[[Anemia]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |CT scan
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |History of [[trauma]]
|-
! rowspan="3" style="padding: 5px 5px; background: #DCDCDC;" align="center" |Gynaecological Causes
! rowspan="2" style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Ovarian cyst|Ovarian Cyst]] Complications
|style="padding: 5px 5px; background: #DCDCDC;" align="center" |Torsion of the cyst
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |RLQ / LLQ
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>+/-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>+/-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |N
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Increased [[ESR]] and [[CRP]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Ultrasound
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Sudden onset sever pain with [[nausea and vomiting]]
|-
|style="padding: 5px 5px; background: #DCDCDC;" align="center" |Cyst rupture
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |RLQ / LLQ
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>+/-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>+/-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |N
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Increased [[ESR]] and [[CRP]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Ultrasound
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Sudden onset sever pain with [[nausea and vomiting]]
|-
!style="padding: 5px 5px; background: #DCDCDC;" align="center" |Pregnancy
|style="padding: 5px 5px; background: #DCDCDC;" align="center" |Ruptured [[ectopic pregnancy]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |RLQ / LLQ
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |<nowiki>-</nowiki>
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |N
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Positive [[pregnancy test]]
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |Ultrasound
|style="padding: 5px 5px; background: #F5F5F5;" align="left" |History of missed period and [[vaginal bleeding]]
|-
|}


==References==
==References==


{{Reflist|2}}
{{Reflist|2}}
[[Category:Needs content]]
[[Category:Arthritis]]
[[Category:Rheumatology]]
[[Category:Gastroenterology]]
[[Category:Immune system disorders]]
[[Category:Genetic disorders]]
[[Category:Inborn errors of metabolism]]
[[Category:Disease]]
{{WH}}
{{WS}}

Latest revision as of 17:46, 1 August 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]

Overview

Familial mediterranean fever must be differentiated from other diseases that cause fever, fatigue, weight loss, arthralgia, myalgia, rash and soft tissue swelling.

Differential diagnosis

Familial mediterranean fever (FMF) should be differentiated from other conditions presenting with fever, fatigue, weight loss, arthralgia, myalgia, rash and soft tissue swelling. The differentials include the following:[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25]

Category of Disease Diseases Signs and symptoms Laboratory findings
Inheritance pattern Fever duration Frequency of attacks Abdominal pain Arthralgia/Arthritis Chest pain Skin rash Myalgia/Body pain Diarrhea/Vomiting Neurologic manifestations Conjunctivitis Aphthous stomatitis Lymphadenopathy Splenomegaly Complete blood count (CBC) C- reactive protein (CRP)
Erythrocyte sedimentation rate (ESR) Other findings Genetic analysis

Autoinflammatory diseases

 Familial mediterranean fever[26][27]
  • 12-72 h
  • Weekly or 3-4 times/year
+ + + + + -/+ -/+ -/+ +
 Hyper IgD with recurrent fever[27][28][29]
  • 3-7 days
  • Every 2-12 weeks
+ + + + + - +/- +/- +/-
 TNF receptor-associated periodic syndrome[30][31]
  • 3-4 weeks
  • Variable
+ + -
  • Migrating rash with deep pain under the areas with the rash
  • Severe pain follows the rash path in a centrifugal pattern
- - + - +/- +
Muckle-Wells Syndrome[32][33]
  • 2-3 days
  • More common during cold seasons
+ + - + + + + - -
  • Cold-triggered attacks
Familial cold urticaria[27][34]
  • 12-24 hours, or longer
  • Common in cold seasons
- + - - - + +/- - -
  • Cold-triggered attacks
Neonatal onset multisystem inflammatory disease[27][35][36]
  • Continuous
  • Common in cold seasons
+ + + + + +/- +
Pyogenic sterile arthritis, pyoderma gangrenosum, acne (Papa syndrome)[37][38]
  • Variable
  • Variable
+/- +/- +/- +/- - - - - -
 Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA)[39][40][41]
  • Unkown
  • 3-6 days
  • Every 21-28 days
+ + - - + + - - + -
  • Unknown
Blau syndrome[42][43]
  • Intermittent or persistent daily fever
  • Variable
+/- + +/- + +/- - + +/- +
Category of Disease Diseases Signs and symptoms Laboratory findings
Fever Fatigue Arthralgia Myalgia Soft tissue swelling/serositis Skin rash Weight loss Dyspnea Sore throat Lymphadenopathy Complete blood count (CBC) Liver function tests (LFTs)

Inflammatory markers

Autoantibodies

Diagnostic tests

Erythrocyte sedimentation rate (ESR) C- reactive protein (CRP) Anti-nuclear antibodies (ANA) Rheumatoid factor (RF) Anti- glomerular basement membrane (anti-GBM) Anti-dsDNA Anti-Jo1/ Anti Mi2 ANCA

Infections

 HIV + + + + +/- - + +/- + /- + - - - - - -
 Herpesviridae + + + + + - - +/- + - - - - - - -
 Measles + + + + - - - + + - - - - - - -
 Viral hepatitis + + - +/- - - +/- - - +/- - - - - - -
 Parvovirus B19 + + + +/- -
  • Slapped cheek rash
- - - + - - - - - -
Infective endocarditis + + + +/- - +/- + - + - - - - - - - Blood cultures, ultrasonography
Borreliosis, Brucellosis, Yersiniosis + + + + - - - - + - - - - - - Serology, PCR
Syphilis and Jarisch-Herxheimer reaction + + + + - - - + +
  • ALT (Uncommon)
  • AST (Uncommon)
- - - - - - Serology, PCR
Toxoplasmosis + + - + - - - + + - - - - - - Serology, PCR

Neoplasia

Malignant lymphoma + + - +/- +/- + + - + - - - - - - CT, PET/CT, Bone marrow examination, lymph node biopsy
Multicentric Castleman disease + + - - + - + + - + - - - - - - - Lymph node biopsy
Angioimmunoblastic T cell lymphoma + + - - - + - - + - - - - - - Lymph node biopsy

Drug hypersensitivity

Drug reaction with eosinophilia and systemic symptoms + + + + +/- - + - - - - - - - - - Eosinophil count, skin biopsy
Autoimmune conditions Systemic lupus erythematosus + + + +/- + + + - +/- + + - + - - Antinuclear autoantibodies
Inflammatory myositis + + - + (weakness > pain) -
  • Macular red rash over the back of the fingers, elbows or knees (Grotton's sign)
  • Macular purpish or reddish rash on the upper chest or back  (Shawl-like, heliotrope rash)
- - - +/- - +/- +/- - - + - Idem, muscle biopsy
Rheumatoid arthritis + + + - + - + - + - +/- +/- - - - - Anti-citrullinated peptids autoantibodies, rheumatoid factor
Systemic vasculitides + + + - + - +/- - +/- - - - +/- - - + ANCA, tissue biopsy, arteriography
Familial Mediterranean fever + + + + + + + (due to pain) - +/- - - - - - - - Familial history, MEFV gene analysis
Mevalonate kinase deficiency + + + + - + - + + - - - - - - - Urinary mevalonic acid, mevalonate kinase analysis
Reactive arthritis + + + - - - + (Aortic insufficiency) - + - - - - - - - HLA B27, magnetic resonance imaging

Miscellaneous

Sarcoidosis + + + - + + + - +
  • Normal ALT, AST
  • ALP ↑ (infiltrative pattern)
- - - - - -
  • Lymph node/Lung biopsy
  • ACE levels
  • FDG-PET

Less common differentials

FMF must be differentiated from other causes of secondary peritonitis

Differentiating FMF from other causes of peritonitis
Disease Prominent clinical findings Lab tests Tratment
Primary peritonitis Spontaneous bacterial peritonitis
Tuberculous peritonitis
Continuous Ambulatory Peritoneal Dialysis (CAPD peritonitis)
Secondary peritonitis Acute bacterial secondary peritonitis
Biliary peritonitis
Tertiary peritonitis
Familial Mediterranean fever (periodic peritonitis, familial paroxysmal polyserositis)
  • Colchicine prevents but does not treat acute attacks.
Granulomatous peritonitis
  • Diagnosed by the demonstration of diagnostic Maltese cross pattern of starch particles.
Sclerosing encapsulating peritonitis
Intraperitoneal abscesses
  • Diagnosed best by CT scan of the abdomen.
  • Treatment consists of prompt and complete CT or US guided drainage of the abscess, control of the primary cause, and adjunctive use of effective antibiotics. Open drainage is reserved for abscesses for which percutaneous drainage is inappropriate or unsuccessful.
Peritoneal mesothelioma
peritoneal carcinomatosis
Classification of acute abdomen based on etiology Presentation Symptoms Signs Diagnosis Comments
Fever Abdominal Pain Jaundice Guarding Rebound Tenderness Bowel sounds Lab Findings Imaging
Common causes of Peritonitis Primary Peritonitis Spontaneous bacterial peritonitis + Diffuse - - - Hypoactive
  • Ascitic fluid PMN>250 cells/mm³
  • Culture: Positive for single organism
Ultrasound for evaluation of liver cirrhosis -
Secondary Peritonitis Perforated gastric and duodenal ulcer + Diffuse - + + N
    • Glucose < 50mg/dl
    • Total protein > 1g/dl
Air under diaphragm in upright CXR Upper GI endoscopy for diagnosis
Acute cholangitis + RUQ + - - N Abnormal LFT Ultrasound shows biliary dilatation Biliary drainage (ERCP) + IV antibiotics
Acute cholecystitis + RUQ + - - Hypoactive Ultrasound shows gallstone and evidence of inflammation Murphy’s sign
Acute pancreatitis + Epigastric +/- - - N Increased amylase / lipase Ultrasound shows evidence of inflammation Pain radiation to back
Acute appendicitis + RLQ - + + Hypoactive Leukocytosis Ultrasound shows evidence of inflammation Nausea & vomiting, decreased appetite
Acute diverticulitis + LLQ +/- + - Hypoactive Leukocytosis CT scan and ultrasound shows evidence of inflammation
Acute salpingitis + LLQ/ RLQ - +/- +/- N Leukocytosis Pelvic ultrasound Vaginal discharge
Hollow Viscous Obstruction Small intestine obstruction - Diffuse - + +/- Hyperactive then absent Leukocytosis Abdominal X ray Nausea & vomiting associated with constipation, abdominal distention
Volvulus - Diffuse - + - Hypoactive Leukocytosis CT scan and abdominal X ray Nausea & vomiting associated with constipation, abdominal distention
Biliary colic - RUQ + - - N Increased bilirubin and alkaline phosphatase Ultrasound Nausea & vomiting
Renal colic - Flank pain - - - N Hematuria CT scan and ultrasound Colicky abdominal pain associated with nausea & vomiting
Vascular Disorders Ischemic causes Mesenteric ischemia +/- Periumbilical - - - Hyperactive Leukocytosis and lactic acidosis CT scan Nausea & vomiting, normal physical examination
Acute ischemic colitis +/- Diffuse - + + Hyperactive then absent Leukocytosis CT scan Nausea & vomiting
Hemorrhagic causes Ruptured abdominal aortic aneurysm - Diffuse - - - N Normal CT scan Unstable hemodynamics
Intra-abdominal or retroperitoneal hemorrhage - Diffuse - - - N Anemia CT scan History of trauma
Gynaecological Causes Ovarian Cyst Complications Torsion of the cyst - RLQ / LLQ - +/- +/- N Increased ESR and CRP Ultrasound Sudden onset sever pain with nausea and vomiting
Cyst rupture - RLQ / LLQ - +/- +/- N Increased ESR and CRP Ultrasound Sudden onset sever pain with nausea and vomiting
Pregnancy Ruptured ectopic pregnancy - RLQ / LLQ - - - N Positive pregnancy test Ultrasound History of missed period and vaginal bleeding

References

  1. Ejilemele AA, Nwauche CA, Ejele OA (December 2007). "Pattern of abnormal liver enzymes in HIV patients presenting at a Nigerian Tertiary Hospital". Niger Postgrad Med J. 14 (4): 306–9. PMID 18163139.
  2. Gøransson LG, Omdal R, Husby G (March 1992). "[Adult-onset Still's disease. Diagnosis, differential diagnosis and treatment]". Tidsskr. Nor. Laegeforen. (in Norwegian). 112 (9): 1155–5. PMID 1579936.
  3. Hatakka A, Klein J, He R, Piper J, Tam E, Walkty A (September 2011). "Acute hepatitis as a manifestation of parvovirus B19 infection". J. Clin. Microbiol. 49 (9): 3422–4. doi:10.1128/JCM.00575-11. PMC 3165617. PMID 21734024.
  4. Yaguchi D, Marui N, Matsuo M (2015). "Three Adult Cases of HPV-B19 Infection with Concomitant Leukopenia and Low Platelet Counts". Clin Med Insights Case Rep. 8: 19–22. doi:10.4137/CCRep.S18085. PMC 4345940. PMID 25780346.
  5. Díaz F, Collazos J (March 2000). "Hepatic dysfunction due to parvovirus B19 infection". J. Infect. Chemother. 6 (1): 63–4. doi:10.1007/s101560000023. PMID 11810534.
  6. "watermark.silverchair.com" (PDF).
  7. Shetty RK, Vivek G, Naha K, Bekkam S (January 2013). "Right-sided infective endocarditis presenting with purpuric skin rash and cardiac failure in a patient without fever". BMJ Case Rep. 2013. doi:10.1136/bcr-2012-007841. PMC 3603787. PMID 23355575.
  8. Aucott JN, Crowder LA, Yedlin V, Kortte KB (2012). "Bull's-Eye and Nontarget Skin Lesions of Lyme Disease: An Internet Survey of Identification of Erythema Migrans". Dermatol Res Pract. 2012: 451727. doi:10.1155/2012/451727. PMC 3485866. PMID 23133445.
  9. Karaali Z, Baysal B, Poturoglu S, Kendir M (May 2011). "Cutaneous manifestations in brucellosis". Indian J Dermatol. 56 (3): 339–40. doi:10.4103/0019-5154.82505. PMC 3132922. PMID 21772606.
  10. La Spada E, Micalizzi A, La Spada M, Quartarano P, Nugara G, Soresi M, Affronti M, Montalto G (September 2008). "[Abnormal liver function in brucellosis]". Infez Med (in Italian). 16 (3): 148–53. PMID 18843212.
  11. French P (January 2007). "Syphilis". BMJ. 334 (7585): 143–7. doi:10.1136/bmj.39085.518148.BE. PMC 1779891. PMID 17235095.
  12. "Syphilis: Review with Emphasis on Clinical, Epidemiologic, and Some Biologic Features".
  13. Baveja S, Garg S, Rajdeo A (March 2014). "Syphilitic hepatitis: an uncommon manifestation of a common disease". Indian J Dermatol. 59 (2): 209. doi:10.4103/0019-5154.127711. PMC 3969699. PMID 24700957.
  14. Mawhorter SD, Effron D, Blinkhorn R, Spagnuolo PJ (May 1992). "Cutaneous manifestations of toxoplasmosis". Clin. Infect. Dis. 14 (5): 1084–8. PMID 1600010.
  15. Flegr J, Prandota J, Sovičková M, Israili ZH (2014). "Toxoplasmosis--a global threat. Correlation of latent toxoplasmosis with specific disease burden in a set of 88 countries". PLoS ONE. 9 (3): e90203. doi:10.1371/journal.pone.0090203. PMC 3963851. PMID 24662942.
  16. Furtado JM, Smith JR, Belfort R, Gattey D, Winthrop KL (July 2011). "Toxoplasmosis: a global threat". J Glob Infect Dis. 3 (3): 281–4. doi:10.4103/0974-777X.83536. PMC 3162817. PMID 21887062.
  17. Ripert C (March 2000). "[Reactive hypereosinophilia in parasitic diseases]". Rev Prat (in French). 50 (6): 602–7. PMID 10808314.
  18. Alvarado-Esquivel C, Torres-Berumen JL, Estrada-Martínez S, Liesenfeld O, Mercado-Suarez MF (May 2011). "Toxoplasma gondii infection and liver disease: a case-control study in a northern Mexican population". Parasit Vectors. 4: 75. doi:10.1186/1756-3305-4-75. PMC 3105944. PMID 21569516.
  19. Han T, Stutzman L (July 1967). "Mode of spread in patients with localized malignant lymphoma". Arch. Intern. Med. 120 (1): 1–7. PMID 5339237.
  20. Saeed-Abdul-Rahman I, Al-Amri AM (September 2012). "Castleman disease". Korean J Hematol. 47 (3): 163–77. doi:10.5045/kjh.2012.47.3.163. PMC 3464333. PMID 23071471.
  21. Saeed-Abdul-Rahman I, Al-Amri AM (September 2012). "Castleman disease". Korean J Hematol. 47 (3): 163–77. doi:10.5045/kjh.2012.47.3.163. PMC 3464333. PMID 23071471.
  22. Papadavid E, Panayiotides I, Dalamaga M, Katoulis A, Economopoulos T, Stavrianeas N (2010). "Cutaneous involvement in angioimmunoblastic T-cell lymphoma". Indian J Dermatol. 55 (3): 279–80. doi:10.4103/0019-5154.70704. PMC 2965920. PMID 21063526.
  23. Brockow K, Przybilla B, Aberer W, Bircher AJ, Brehler R, Dickel H, Fuchs T, Jakob T, Lange L, Pfützner W, Mockenhaupt M, Ott H, Pfaar O, Ring J, Sachs B, Sitter H, Trautmann A, Treudler R, Wedi B, Worm M, Wurpts G, Zuberbier T, Merk HF (2015). "Guideline for the diagnosis of drug hypersensitivity reactions: S2K-Guideline of the German Society for Allergology and Clinical Immunology (DGAKI) and the German Dermatological Society (DDG) in collaboration with the Association of German Allergologists (AeDA), the German Society for Pediatric Allergology and Environmental Medicine (GPA), the German Contact Dermatitis Research Group (DKG), the Swiss Society for Allergy and Immunology (SGAI), the Austrian Society for Allergology and Immunology (ÖGAI), the German Academy of Allergology and Environmental Medicine (DAAU), the German Center for Documentation of Severe Skin Reactions and the German Federal Institute for Drugs and Medical Products (BfArM)". Allergo J Int. 24 (3): 94–105. doi:10.1007/s40629-015-0052-6. PMC 4479479. PMID 26120552.
  24. Medlej-Hashim M, Loiselet J, Lefranc G, Mégarbané A (2004). "[Familial Mediterranean Fever (FMF): from diagnosis to treatment]". Sante (in French). 14 (4): 261–6. PMID 15745878.
  25. Zhang S (May 2016). "Natural history of mevalonate kinase deficiency: a literature review". Pediatr Rheumatol Online J. 14 (1): 30. doi:10.1186/s12969-016-0091-7. PMC 4855321. PMID 27142780.
  26. M. Medlej-Hashim, I. Petit, S. Adib, E. Chouery, N. Salem, V. Delague, M. Rawashdeh, I. Mansour, G. Lefranc, R. Naman, J. Loiselet, J. C. Lecron, J. L. Serre & A. Megarbane (2001). "Familial Mediterranean Fever: association of elevated IgD plasma levels with specific MEFV mutations". European journal of human genetics : EJHG. 9 (11): 849–854. doi:10.1038/sj.ejhg.5200725. PMID 11781702. Unknown parameter |month= ignored (help)
  27. 27.0 27.1 27.2 27.3 Kastner, D. L. (2005). "Hereditary Periodic Fever Syndromes". Hematology. 2005 (1): 74–81. doi:10.1182/asheducation-2005.1.74. ISSN 1520-4391.
  28. Kraus, Courtney L; Culican, Susan M (2009). "Nummular keratopathy in a patient with Hyper-IgD Syndrome". Pediatric Rheumatology. 7 (1). doi:10.1186/1546-0096-7-14. ISSN 1546-0096.
  29. Mulders-Manders, C. M.; Simon, A. (2015). "Hyper-IgD syndrome/mevalonate kinase deficiency: what is new?". Seminars in Immunopathology. 37 (4): 371–376. doi:10.1007/s00281-015-0492-6. ISSN 1863-2297.
  30. Toro, Jorge R.; Aksentijevich, Ivona; Hull, Keith; Dean, Jane; Kastner, Daniel L. (2000). "Tumor Necrosis Factor Receptor–Associated Periodic Syndrome". Archives of Dermatology. 136 (12). doi:10.1001/archderm.136.12.1487. ISSN 0003-987X.
  31. Lachmann, H J; Papa, R; Gerhold, K; Obici, L; Touitou, I; Cantarini, L; Frenkel, J; Anton, J; Kone-Paut, I; Cattalini, M; Bader-Meunier, B; Insalaco, A; Hentgen, V; Merino, R; Modesto, C; Toplak, N; Berendes, R; Ozen, S; Cimaz, R; Jansson, A; Brogan, P A; Hawkins, P N; Ruperto, N; Martini, A; Woo, P; Gattorno, M (2014). "The phenotype of TNF receptor-associated autoinflammatory syndrome (TRAPS) at presentation: a series of 158 cases from the Eurofever/EUROTRAPS international registry". Annals of the Rheumatic Diseases. 73 (12): 2160–2167. doi:10.1136/annrheumdis-2013-204184. ISSN 0003-4967.
  32. Hawkins, Philip N.; Lachmann, Helen J.; Aganna, Ebun; McDermott, Michael F. (2004). "Spectrum of clinical features in Muckle-Wells syndrome and response to anakinra". Arthritis & Rheumatism. 50 (2): 607–612. doi:10.1002/art.20033. ISSN 0004-3591.
  33. Ahmadi, Neda; Brewer, Carmen C.; Zalewski, Christopher; King, Kelly A.; Butman, John A.; Plass, Nicole; Henderson, Cailin; Goldbach-Mansky, Raphaela; Kim, H. Jeffrey (2011). "Cryopyrin-Associated Periodic Syndromes". Otolaryngology–Head and Neck Surgery. 145 (2): 295–302. doi:10.1177/0194599811402296. ISSN 0194-5998.
  34. Stych, Beate; Dobrovolny, Diana (2008). "Familial cold auto-inflammatory syndrome (FCAS): characterization of symptomatology and impact on patients' lives". Current Medical Research and Opinion. 24 (6): 1577–1582. doi:10.1185/03007990802081543. ISSN 0300-7995.
  35. Goldbach-Mansky, Raphaela; Dailey, Natalie J.; Canna, Scott W.; Gelabert, Ana; Jones, Janet; Rubin, Benjamin I.; Kim, H. Jeffrey; Brewer, Carmen; Zalewski, Christopher; Wiggs, Edythe; Hill, Suvimol; Turner, Maria L.; Karp, Barbara I.; Aksentijevich, Ivona; Pucino, Frank; Penzak, Scott R.; Haverkamp, Margje H.; Stein, Leonard; Adams, Barbara S.; Moore, Terry L.; Fuhlbrigge, Robert C.; Shaham, Bracha; Jarvis, James N.; O'Neil, Kathleen; Vehe, Richard K.; Beitz, Laurie O.; Gardner, Gregory; Hannan, William P.; Warren, Robert W.; Horn, William; Cole, Joe L.; Paul, Scott M.; Hawkins, Philip N.; Pham, Tuyet Hang; Snyder, Christopher; Wesley, Robert A.; Hoffmann, Steven C.; Holland, Steven M.; Butman, John A.; Kastner, Daniel L. (2006). "Neonatal-Onset Multisystem Inflammatory Disease Responsive to Interleukin-1β Inhibition". New England Journal of Medicine. 355 (6): 581–592. doi:10.1056/NEJMoa055137. ISSN 0028-4793.
  36. Kim, Hanna; Montealegre Sanchez, Gina A.; Chapelle, Dawn C.; Plass, Nicole; Dwyer, Andrew; Goldbach-Mansky, Raphaela; Hill, Suvimol (2014). "A80: Skeletal Features of Neonatal-Onset Multisystem Inflammatory Disease (NOMID) on Anakinra Treatment: Long-Term Follow-up". Arthritis & Rheumatology. 66: S113–S113. doi:10.1002/art.38496. ISSN 2326-5191.
  37. Yeon, Howard B.; Lindor, Noralane M.; Seidman, J.G.; Seidman, Christine E. (2000). "Pyogenic Arthritis, Pyoderma Gangrenosum, and Acne Syndrome Maps to Chromosome 15q". The American Journal of Human Genetics. 66 (4): 1443–1448. doi:10.1086/302866. ISSN 0002-9297.
  38. Schellevis, M. A.; Stoffels, M.; Hoppenreijs, E. P. A. H.; Bodar, E.; Simon, A.; van der Meer, J. W. M. (2011). "Variable expression and treatment of PAPA syndrome". Annals of the Rheumatic Diseases. 70 (6): 1168–1170. doi:10.1136/ard.2009.126185. ISSN 0003-4967.
  39. Vanoni, Federica; Federici, Silvia; Antón, Jordi; Barron, Karyl S.; Brogan, Paul; De Benedetti, Fabrizio; Dedeoglu, Fatma; Demirkaya, Erkan; Hentgen, Veronique; Kallinich, Tilmann; Laxer, Ronald; Russo, Ricardo; Toplak, Natasa; Uziel, Yosef; Martini, Alberto; Ruperto, Nicolino; Gattorno, Marco; Hofer, Michael (2018). "An international delphi survey for the definition of the variables for the development of new classification criteria for periodic fever aphtous stomatitis pharingitis cervical adenitis (PFAPA)". Pediatric Rheumatology. 16 (1). doi:10.1186/s12969-018-0246-9. ISSN 1546-0096.
  40. Cattalini, Marco; Soliani, Martina; Rigante, Donato; Lopalco, Giuseppe; Iannone, Florenzo; Galeazzi, Mauro; Cantarini, Luca (2015). "Basic Characteristics of Adults with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenopathy Syndrome in Comparison with the Typical Pediatric Expression of Disease". Mediators of Inflammation. 2015: 1–11. doi:10.1155/2015/570418. ISSN 0962-9351.
  41. Gattorno, M.; Caorsi, R.; Meini, A.; Cattalini, M.; Federici, S.; Zulian, F.; Cortis, E.; Calcagno, G.; Tommasini, A.; Consolini, R.; Simonini, G.; Pelagatti, M. A.; Baldi, M.; Ceccherini, I.; Plebani, A.; Frenkel, J.; Sormani, M. P.; Martini, A. (2009). "Differentiating PFAPA Syndrome From Monogenic Periodic Fevers". PEDIATRICS. 124 (4): e721–e728. doi:10.1542/peds.2009-0088. ISSN 0031-4005.
  42. Rosé, Carlos D.; Aróstegui, Juan I.; Martin, Tammy M.; Espada, Graciela; Scalzi, Lisabeth; Yagüe, Jordi; Rosenbaum, James T.; Modesto, Consuelo; Cristina Arnal, Maria; Merino, Rosa; García-Consuegra, Julia; Carballo Silva, María Antonia; Wouters, Carine H. (2009). "NOD2-Associated pediatric granulomatous arthritis, an expanding phenotype: Study of an international registry and a national cohort in spain". Arthritis & Rheumatism. 60 (6): 1797–1803. doi:10.1002/art.24533. ISSN 0004-3591.
  43. Kim, Woojoong; Park, Eujin; Ahn, Yo Han; Lee, Jiwon M.; Kang, Hee Gyung; Kim, Byung Joo; Ha, Il-Soo; Cheong, Hae Il (2016). "A familial case of Blau syndrome caused by a novelNOD2genetic mutation". Korean Journal of Pediatrics. 59 (Suppl 1): S5. doi:10.3345/kjp.2016.59.11.S5. ISSN 1738-1061.