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{{Familial mediterranean fever}}
{{Familial mediterranean fever}}
{{CMG}}
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{{CMG}}; {{AE}} {{Sahar}}
==Overview==
==Overview==
The diagnosis is clinically made on the basis of the history of typical attacks, especially in patients from the ethnic groups in which FMF is more highly prevalent.
The hallmark of [[familial Mediterranean fever]] is [[periodic fever]] and [[serositis]]. A positive history of [[periodic fever]] lasting 1 to 3 days and [[serositis]] is suggestive of [[FMF]]. Common [[symptoms]] of [[Familial mediterranean fever|familial Mediterranean fever]] include [[abdominal pain]], [[Periodic fever|episodic fever]], [[arthralgia]], [[chest pain]], [[myalgia]], [[vomiting]], and [[fatigue]].
==History and Symptoms==
==History and Symptoms==
There are seven types of attacks. 90% of all patients have their first attacks before they are 20 years old. All develop over 2-4 hours and last anytime between 6 hours and 4 days. Most attacks involve [[fever]]:<ref name=Livneh>Livneh A, Langevitz P. Diagnostic and treatment concerns in familial Mediterranean fever. ''Baillieres Best Pract Res Clin Rheumatol'' 2000;14(3):477-98. PMID 10985982.</ref>
*The hallmark of [[Familial mediterranean fever|familial Mediterranean fever]] is [[periodic fever]] and [[serositis]]. A positive history of [[periodic fever]] lasting 1 to 3 days and [[serositis]] is suggestive of [[FMF]].
# Abdominal attacks, featuring [[abdominal pain]] affecting the whole abdomen with all signs of [[acute abdomen]] (e.g. [[appendicitis]]). They occur in 95% of all patients and may lead to unnecessary [[laparotomy]]. Incomplete attacks, with local tenderness and normal blood tests, have been reported.
===History===
# Joint attacks, occurring in large joints, mainly of the legs. Usually, only one joint is affected. 75% of all FMF patients experience joint attacks.
[[Patients]] with [[Familial mediterranean fever|familial Mediterranean fever]] may have a positive history of:<ref name="pmid5340644">{{cite journal |vauthors=Sohar E, Gafni J, Pras M, Heller H |title=Familial Mediterranean fever. A survey of 470 cases and review of the literature |journal=Am. J. Med. |volume=43 |issue=2 |pages=227–53 |date=August 1967 |pmid=5340644 |doi= |url=}}</ref><ref name="pmid28828621">{{cite journal |vauthors=Barut K, Sahin S, Adrovic A, Sinoplu AB, Yucel G, Pamuk G, Aydın AK, Dasdemir S, Turanlı ET, Buyru N, Kasapcopur O |title=Familial Mediterranean fever in childhood: a single-center experience |journal=Rheumatol. Int. |volume=38 |issue=1 |pages=67–74 |date=January 2018 |pmid=28828621 |doi=10.1007/s00296-017-3796-0 |url=}}</ref>
# Chest attacks with [[pleuritis]] (inflammation of the [[pleura]]l lining) and [[pericarditis]] (inflammation of the [[pericardium]]). Pleuritis occurs in 40%, but pericarditis is rare.
*[[Periodic fever]]
# [[Scrotum|Scrotal]] attacks due to inflammation of the [[tunica vaginalis]]. This occurs in up to 5% and may be mistaken for acute scrotum. (i.e. [[testicular torsion]])
*Recurrent attacks of [[abdominal pain]]
# [[Myalgia]] (rare in isolation)
*Recurrent attacks of [[chest pain]]
# [[Erysipelas|Erysipeloid]] (a skin reaction on the legs, rare in isolation)
 
# [[Fever]] without any symptoms. (25%)
===Common Symptoms===
Common [[symptoms]] of [[Familial mediterranean fever|familial Mediterranean fever]] include:<ref name="FedericiSormani2015">{{cite journal|last1=Federici|first1=Silvia|last2=Sormani|first2=Maria Pia|last3=Ozen|first3=Seza|last4=Lachmann|first4=Helen J|last5=Amaryan|first5=Gayane|last6=Woo|first6=Patricia|last7=Koné-Paut|first7=Isabelle|last8=Dewarrat|first8=Natacha|last9=Cantarini|first9=Luca|last10=Insalaco|first10=Antonella|last11=Uziel|first11=Yosef|last12=Rigante|first12=Donato|last13=Quartier|first13=Pierre|last14=Demirkaya|first14=Erkan|last15=Herlin|first15=Troels|last16=Meini|first16=Antonella|last17=Fabio|first17=Giovanna|last18=Kallinich|first18=Tilmann|last19=Martino|first19=Silvana|last20=Butbul|first20=Aviel Yonatan|last21=Olivieri|first21=Alma|last22=Kuemmerle-Deschner|first22=Jasmin|last23=Neven|first23=Benedicte|last24=Simon|first24=Anna|last25=Ozdogan|first25=Huri|last26=Touitou|first26=Isabelle|last27=Frenkel|first27=Joost|last28=Hofer|first28=Michael|last29=Martini|first29=Alberto|last30=Ruperto|first30=Nicolino|last31=Gattorno|first31=Marco|title=Evidence-based provisional clinical classification criteria for autoinflammatory periodic fevers|journal=Annals of the Rheumatic Diseases|volume=74|issue=5|year=2015|pages=799–805|issn=0003-4967|doi=10.1136/annrheumdis-2014-206580}}</ref><ref>{{cite journal|title=2019 CIS Annual Meeting: Immune Deficiency & Dysregulation North American Conference|journal=Journal of Clinical Immunology|volume=39|issue=S1|year=2019|pages=1–151|issn=0271-9142|doi=10.1007/s10875-019-00597-5}}</ref>
*[[Abdominal pain]]
*[[Periodic fever|Episodic fever]]
*[[Arthralgia]]
*[[Chest pain]]
*[[Myalgia]]
*[[Vomiting]]
*[[Fatigue]]


===Less Common Symptoms===
Less common [[symptoms]] of [[Familial mediterranean fever|familial Mediterranean fever]] include:<ref name="FedericiSormani2015">{{cite journal|last1=Federici|first1=Silvia|last2=Sormani|first2=Maria Pia|last3=Ozen|first3=Seza|last4=Lachmann|first4=Helen J|last5=Amaryan|first5=Gayane|last6=Woo|first6=Patricia|last7=Koné-Paut|first7=Isabelle|last8=Dewarrat|first8=Natacha|last9=Cantarini|first9=Luca|last10=Insalaco|first10=Antonella|last11=Uziel|first11=Yosef|last12=Rigante|first12=Donato|last13=Quartier|first13=Pierre|last14=Demirkaya|first14=Erkan|last15=Herlin|first15=Troels|last16=Meini|first16=Antonella|last17=Fabio|first17=Giovanna|last18=Kallinich|first18=Tilmann|last19=Martino|first19=Silvana|last20=Butbul|first20=Aviel Yonatan|last21=Olivieri|first21=Alma|last22=Kuemmerle-Deschner|first22=Jasmin|last23=Neven|first23=Benedicte|last24=Simon|first24=Anna|last25=Ozdogan|first25=Huri|last26=Touitou|first26=Isabelle|last27=Frenkel|first27=Joost|last28=Hofer|first28=Michael|last29=Martini|first29=Alberto|last30=Ruperto|first30=Nicolino|last31=Gattorno|first31=Marco|title=Evidence-based provisional clinical classification criteria for autoinflammatory periodic fevers|journal=Annals of the Rheumatic Diseases|volume=74|issue=5|year=2015|pages=799–805|issn=0003-4967|doi=10.1136/annrheumdis-2014-206580}}</ref>
*[[Sore throat]]
*[[Diarrhea]]
*[[Skin rash]]
*[[Headache]]
*[[Aphthous stomatitis]]
*[[Lymphadenopathy|Painful lymph node]]
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
[[Category:Needs content]]


[[Category:Arthritis]]
[[Category:Arthritis]]

Latest revision as of 03:45, 4 June 2019


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

Overview

The hallmark of familial Mediterranean fever is periodic fever and serositis. A positive history of periodic fever lasting 1 to 3 days and serositis is suggestive of FMF. Common symptoms of familial Mediterranean fever include abdominal pain, episodic fever, arthralgia, chest pain, myalgia, vomiting, and fatigue.

History and Symptoms

History

Patients with familial Mediterranean fever may have a positive history of:[1][2]

Common Symptoms

Common symptoms of familial Mediterranean fever include:[3][4]

Less Common Symptoms

Less common symptoms of familial Mediterranean fever include:[3]

References

  1. Sohar E, Gafni J, Pras M, Heller H (August 1967). "Familial Mediterranean fever. A survey of 470 cases and review of the literature". Am. J. Med. 43 (2): 227–53. PMID 5340644.
  2. Barut K, Sahin S, Adrovic A, Sinoplu AB, Yucel G, Pamuk G, Aydın AK, Dasdemir S, Turanlı ET, Buyru N, Kasapcopur O (January 2018). "Familial Mediterranean fever in childhood: a single-center experience". Rheumatol. Int. 38 (1): 67–74. doi:10.1007/s00296-017-3796-0. PMID 28828621.
  3. 3.0 3.1 Federici, Silvia; Sormani, Maria Pia; Ozen, Seza; Lachmann, Helen J; Amaryan, Gayane; Woo, Patricia; Koné-Paut, Isabelle; Dewarrat, Natacha; Cantarini, Luca; Insalaco, Antonella; Uziel, Yosef; Rigante, Donato; Quartier, Pierre; Demirkaya, Erkan; Herlin, Troels; Meini, Antonella; Fabio, Giovanna; Kallinich, Tilmann; Martino, Silvana; Butbul, Aviel Yonatan; Olivieri, Alma; Kuemmerle-Deschner, Jasmin; Neven, Benedicte; Simon, Anna; Ozdogan, Huri; Touitou, Isabelle; Frenkel, Joost; Hofer, Michael; Martini, Alberto; Ruperto, Nicolino; Gattorno, Marco (2015). "Evidence-based provisional clinical classification criteria for autoinflammatory periodic fevers". Annals of the Rheumatic Diseases. 74 (5): 799–805. doi:10.1136/annrheumdis-2014-206580. ISSN 0003-4967.
  4. "2019 CIS Annual Meeting: Immune Deficiency & Dysregulation North American Conference". Journal of Clinical Immunology. 39 (S1): 1–151. 2019. doi:10.1007/s10875-019-00597-5. ISSN 0271-9142.