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{{Whipple's disease}}
{{Whipple's disease}}
{{CMG}}; {{AE}} {{BHA}}
{{CMG}}; {{AE}} {{SSH}}; {{BHA}}


==Overview==
==Overview==
*Patients with Whipple's disease have varied presentation.
Patients with Whipple's disease have various presentations. Most common symptoms of the classic Whipple's disease include [[joint pain]], [[weight loss]], [[diarrhea]], and [[abdominal pain]]. Other organ systems can be involved in Whipple's disease including [[central nervous system]], [[cardiac]] system, [[renal]] system, [[skeletal]], [[muscles]] and [[pulmonary]] system.
*Most common symptoms of Classical Whipple's disease include joint pain weight loss diarrhea and arthropathy. Various other organ systems can be involved in Whipple's disease including central nervous system, cardiac system, renal system, skeletal muscles and pulmonary system.  


==History and Symptoms==
==History and Symptoms==
[[image:Whipple's disease1.gif|center|frame]]
=== History ===
Obtaining history is important to find risk factors for the Whipple's disease. Other differentials specifically those which cause [[malabsorption]] must be ruled out. Specific areas of focus when obtaining the history, are outlined below:<ref name="pmid11432814" /><ref name="pmid17202456" /><ref name="pmid18291339" /><ref name="MarthMoos2016" /><ref name="Schoniger-HekelePetermann2007" /><ref name="BurešKopáčová2013" /><ref name="DutlyAltwegg2001" /><ref name="DolmansBoel2017" /><ref name="pmid18159328" /><ref name="FenollarLagier2014" /><ref name="KeitaBrouqui2013" /><ref name="SchwartzmanSchwartzman2013" />
* Onset, duration and progression of [[symptoms]]
* History of consuming contaminated water or vegetables
* History of access to proper [[sanitation]] and toilets
* History of living in shelters
* History of similar [[symptoms]] in a family
* Medical history of [[immunosuppression]] therapy
* History of [[HIV infection]]
===Common Symptoms===
Whipple's disease is a systemic disease that involves multiple [[organs]]. Many of the patients present with [[gastrointestinal]] symptoms, while others presents with localized involvement of different [[organs]].<ref name="pmid11432814">{{cite journal| author=Dutly F, Altwegg M| title=Whipple's disease and "Tropheryma whippelii". | journal=Clin Microbiol Rev | year= 2001 | volume= 14 | issue= 3 | pages= 561-83 | pmid=11432814 | doi=10.1128/CMR.14.3.561-583.2001 | pmc=88990 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11432814  }} </ref><ref name="pmid17202456">{{cite journal| author=Fenollar F, Puéchal X, Raoult D| title=Whipple's disease. | journal=N Engl J Med | year= 2007 | volume= 356 | issue= 1 | pages= 55-66 | pmid=17202456 | doi=10.1056/NEJMra062477 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17202456  }} </ref><ref name="pmid18291339">{{cite journal |vauthors=Schneider T, Moos V, Loddenkemper C, Marth T, Fenollar F, Raoult D |title=Whipple's disease: new aspects of pathogenesis and treatment |journal=Lancet Infect Dis |volume=8 |issue=3 |pages=179–90 |year=2008 |pmid=18291339 |doi=10.1016/S1473-3099(08)70042-2 |url=}}</ref><ref name="MarthMoos2016">{{cite journal|last1=Marth|first1=Thomas|last2=Moos|first2=Verena|last3=Müller|first3=Christian|last4=Biagi|first4=Federico|last5=Schneider|first5=Thomas|title=Tropheryma whipplei infection and Whipple's disease|journal=The Lancet Infectious Diseases|volume=16|issue=3|year=2016|pages=e13–e22|issn=14733099|doi=10.1016/S1473-3099(15)00537-X}}</ref><ref name="Schoniger-HekelePetermann2007">{{cite journal|last1=Schoniger-Hekele|first1=M.|last2=Petermann|first2=D.|last3=Weber|first3=B.|last4=Muller|first4=C.|title=Tropheryma whipplei in the Environment: Survey of Sewage Plant Influxes and Sewage Plant Workers|journal=Applied and Environmental Microbiology|volume=73|issue=6|year=2007|pages=2033–2035|issn=0099-2240|doi=10.1128/AEM.02335-06}}</ref><ref name="BurešKopáčová2013">{{cite journal|last1=Bureš|first1=Jan|last2=Kopáčová|first2=Marcela|last3=Douda|first3=Tomáš|last4=Bártová|first4=Jolana|last5=Tomš|first5=Jan|last6=Rejchrt|first6=Stanislav|last7=Tachecí|first7=Ilja|title=Whipple’s Disease: Our Own Experience and Review of the Literature|journal=Gastroenterology Research and Practice|volume=2013|year=2013|pages=1–10|issn=1687-6121|doi=10.1155/2013/478349}}</ref><ref name="DutlyAltwegg2001">{{cite journal|last1=Dutly|first1=F.|last2=Altwegg|first2=M.|title=Whipple's Disease and "Tropheryma whippelii"|journal=Clinical Microbiology Reviews|volume=14|issue=3|year=2001|pages=561–583|issn=0893-8512|doi=10.1128/CMR.14.3.561-583.2001}}</ref><ref name="DolmansBoel2017">{{cite journal|last1=Dolmans|first1=Ruben A. V.|last2=Boel|first2=C. H. Edwin|last3=Lacle|first3=Miangela M.|last4=Kusters|first4=Johannes G.|title=Clinical Manifestations, Treatment, and Diagnosis of Tropheryma whipplei Infections|journal=Clinical Microbiology Reviews|volume=30|issue=2|year=2017|pages=529–555|issn=0893-8512|doi=10.1128/CMR.00033-16}}</ref><ref name="pmid18159328">{{cite journal |vauthors=Conly JM, Johnston BL |title=Rare but not so rare: The evolving spectrum of Whipple's disease |journal=Can J Infect Dis |volume=12 |issue=3 |pages=133–5 |year=2001 |pmid=18159328 |pmc=2094813 |doi= |url=}}</ref><ref name="FenollarLagier2014">{{cite journal|last1=Fenollar|first1=Florence|last2=Lagier|first2=Jean-Christophe|last3=Raoult|first3=Didier|title=Tropheryma whipplei and Whipple's disease|journal=Journal of Infection|volume=69|issue=2|year=2014|pages=103–112|issn=01634453|doi=10.1016/j.jinf.2014.05.008}}</ref><ref name="KeitaBrouqui2013">{{cite journal|last1=Keita|first1=Alpha Kabinet|last2=Brouqui|first2=Philippe|last3=Badiaga|first3=Sékéné|last4=Benkouiten|first4=Samir|last5=Ratmanov|first5=Pavel|last6=Raoult|first6=Didier|last7=Fenollar|first7=Florence|title=Tropheryma whipplei prevalence strongly suggests human transmission in homeless shelters|journal=International Journal of Infectious Diseases|volume=17|issue=1|year=2013|pages=e67–e68|issn=12019712|doi=10.1016/j.ijid.2012.05.1033}}</ref><ref name="SchwartzmanSchwartzman2013">{{cite journal|last1=Schwartzman|first1=Sergio|last2=Schwartzman|first2=Monica|title=Whipple's Disease|journal=Rheumatic Disease Clinics of North America|volume=39|issue=2|year=2013|pages=313–321|issn=0889857X|doi=10.1016/j.rdc.2013.03.005}}</ref>
==== Non specific symptoms ====
* [[Fever]]
* [[Weight loss]]
* [[Fatigue]]
* [[Night sweats]]


== Common Symptoms[edit | edit source] ==
====Gastrointestinal system====
* [[Diarrhea]]: The most common complaint
* [[Steatorrhea]]
* [[Abdominal pain]]
* [[Edema]]


====Articular system====
* Intermittent migratory [[arthralgias]]: The second most common complaint
* [[Arthritis]] affecting peripheral [[joints]], such as [[knees]], [[elbows]], [[wrists]], and [[shoulders]]
* Multiple [[joint]] involvement


* Gastrointestinal system : Patients with Classical Whipple's Disease often present with weight loss with diarrhea. [[Diarrhea]] is the most common complain in the patients with Whipple's disease and it is associated with Steatorrhea. Other gastrointestinal symptoms may be often seen like abdominal pain, occult mucosal bleeding, ascites, hepatospleenomegaly and occasionally hepatitis. <ref name="La ScolaAltwegg2001">{{cite journal|last1=La Scola|first1=B|last2=Altwegg|first2=M|last3=Mallet|first3=M N|last4=Fournier|first4=P E|last5=Fenollar|first5=F|last6=Raoult|first6=D|title=Description of Tropheryma whipplei gen. nov., sp. nov., the Whipple's disease bacillus.|journal=International Journal of Systematic and Evolutionary Microbiology|volume=51|issue=4|year=2001|pages=1471–1479|issn=1466-5026|doi=10.1099/00207713-51-4-1471}}</ref>
=== Less Common Symptoms ===
* Articular system : Joint involvement is another predominant complain in patients with Whipple's disease. It is second most commonly involved system after the gastrointestinal system.  Patients may present with intermittent migratory arthralgias and/or arthritis often affecting peripheral joints like knees elbows wrists etc.  Multiple joint involvement is common. Most attacks are acute, rarely chronic arthritis may be destructive.
====Central nervous system symptoms====
* Central nervous system : CNS involvement in Whipple's disease most often presents as asymptomatic involvement with detection of T. whipplei DNA in CSF. In patients with symptomatic disease there might be focal CNS involvement which determine the varied clinical symptoms like dementia, cerebral ataxia,hemiparesis, personality changes, opthalmoplegia, nystagmus etc. About fifty percent patients may have supranuclear opthalmoplegia at presentation and twenty five percent of the patients may present with myoclonus. On rare occasion patients may present with hypothalmic involvement seen as polydipsia, hyperphagia, change in libido and sleep wake cycle.The pathognomic abnormalities in Whipple's are eye movement abnormalities Oculomasticatory myorhythmia (OMM) and oculofacial-skeletal myorhythmia (OSFM). CNS involvement usually has poor prognosis.
* Asymptomatic involvement with detection of [[Tropheryma whipplei|''Tropheryma whipplei'']] [[DNA]] in [[CSF]]: The most common finding
* Focal [[CNS]] involvement:
** [[Forgetfulness]]
** [[Hemiparesis]]
** [[Personality changes]]
** Progressive [[cognitive]] decline
** [[Nystagmus]]
** Twitching of a [[muscle]] 
* [[Hypothalamic]] involvement (rarely)
** [[Polydipsia]]
** [[Hyperphagia]]
** Change in [[libido]]
** Change in sleep wake cycle
* [[Eye movement]] abnormalities (pathognomic)
* [[CNS]] involvement usually has poor prognosis.  
====Ocular symptoms====
[[Ocular]] involvement is rare and is often seen in patients with [[CNS]] disease. It can involve:
* [[Eye pain]]
* [[Eye redness]]
* Decreased [[vision]]
* [[Diplopia]]


== Less Common Symptoms[edit | edit source] ==
====Skeletal muscle symptoms====
* [[Myalgia]]
* [[Muscle cramps]]


====Cardiopulmonary symptoms====
* [[Chest pain]]: It could be [[pleuritic]]
* [[Shortness of breath]]
* [[Palpitation|Palpitations]]
* Chronic [[cough]]
* [[Cyanosis]]
* [[Sudden cardiac death]]


* Ocular involvement in Whipple's disease is rare and can involve uveitis, retinitis, vitritis, retrobulbar neuritis, papilledema. Ocular involvement is often seen in patients with CNS disease.
====Cutaneous symptoms====
* Skeletal muscle involvement in Whipples disease is seen as myalgias and muscle cramps
* [[Skin]] color changes
* Some patients may present with cardiac involvement which has varied presentation ranging from Pericarditis,  Myocarditis, heart failure or Sudden Cardiac death. Endocarditis due to Whipple's disease is associated with negative blood cultures. Endocarditis is often preceeded by arthritis and arthralgias.
* Yellowish discoloration of the [[skin]]
* Pulmonary involvement in classic Whipple’s disease may manifest as pleuritic chest pain, non productive cough and dyspnea. Chest x-ray often shows pleural effusion, pulmonary infiltration, or granulomatous mediastinal adenopathy. Often abdominal lymph nodes may be involved.
 
* Hematological finding in Whipple's disease is Anemia which may be due to malabsorption or mucointestinal bleeding.
====Renal symptoms====
* Classical cutaneous finding reported in Whipple's disease is Melanoderma. this is less frequently reported these days as Whipple's disease is increasingly recognized early.
* [[Renal]] involvement is late in course, may be seen rarely.
* Late in course renal involvement may be seen rarely.


==References==
==References==
http://www.nejm.org/doi/full/10.1056/NEJMra062477
{{Reflist|2}}
 
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http://citeseerx.ist.psu.edu/viewdoc/download?doi=10.1.1.320.992&rep=rep1&type=pdf<nowiki/>{{Reflist|2}}
 
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Latest revision as of 00:44, 30 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]; Bhagyashree Deshmankar M.B.B.S.[3]

Overview

Patients with Whipple's disease have various presentations. Most common symptoms of the classic Whipple's disease include joint pain, weight loss, diarrhea, and abdominal pain. Other organ systems can be involved in Whipple's disease including central nervous system, cardiac system, renal system, skeletal, muscles and pulmonary system.

History and Symptoms

History

Obtaining history is important to find risk factors for the Whipple's disease. Other differentials specifically those which cause malabsorption must be ruled out. Specific areas of focus when obtaining the history, are outlined below:[1][2][3][4][5][6][7][8][9][10][11][12]

  • Onset, duration and progression of symptoms
  • History of consuming contaminated water or vegetables
  • History of access to proper sanitation and toilets
  • History of living in shelters
  • History of similar symptoms in a family
  • Medical history of immunosuppression therapy
  • History of HIV infection

Common Symptoms

Whipple's disease is a systemic disease that involves multiple organs. Many of the patients present with gastrointestinal symptoms, while others presents with localized involvement of different organs.[1][2][3][4][5][6][7][8][9][10][11][12]

Non specific symptoms

Gastrointestinal system

Articular system

Less Common Symptoms

Central nervous system symptoms

Ocular symptoms

Ocular involvement is rare and is often seen in patients with CNS disease. It can involve:

Skeletal muscle symptoms

Cardiopulmonary symptoms

Cutaneous symptoms

  • Skin color changes
  • Yellowish discoloration of the skin

Renal symptoms

  • Renal involvement is late in course, may be seen rarely.

References

  1. ↑ 1.0 1.1 Dutly F, Altwegg M (2001). "Whipple's disease and "Tropheryma whippelii"". Clin Microbiol Rev. 14 (3): 561–83. doi:10.1128/CMR.14.3.561-583.2001. PMC 88990. PMID 11432814.
  2. ↑ 2.0 2.1 Fenollar F, Puéchal X, Raoult D (2007). "Whipple's disease". N Engl J Med. 356 (1): 55–66. doi:10.1056/NEJMra062477. PMID 17202456.
  3. ↑ 3.0 3.1 Schneider T, Moos V, Loddenkemper C, Marth T, Fenollar F, Raoult D (2008). "Whipple's disease: new aspects of pathogenesis and treatment". Lancet Infect Dis. 8 (3): 179–90. doi:10.1016/S1473-3099(08)70042-2. PMID 18291339.
  4. ↑ 4.0 4.1 Marth, Thomas; Moos, Verena; Müller, Christian; Biagi, Federico; Schneider, Thomas (2016). "Tropheryma whipplei infection and Whipple's disease". The Lancet Infectious Diseases. 16 (3): e13–e22. doi:10.1016/S1473-3099(15)00537-X. ISSN 1473-3099.
  5. ↑ 5.0 5.1 Schoniger-Hekele, M.; Petermann, D.; Weber, B.; Muller, C. (2007). "Tropheryma whipplei in the Environment: Survey of Sewage Plant Influxes and Sewage Plant Workers". Applied and Environmental Microbiology. 73 (6): 2033–2035. doi:10.1128/AEM.02335-06. ISSN 0099-2240.
  6. ↑ 6.0 6.1 Bureš, Jan; Kopáčová, Marcela; Douda, Tomáš; Bártová, Jolana; Tomš, Jan; Rejchrt, Stanislav; Tachecí, Ilja (2013). "Whipple's Disease: Our Own Experience and Review of the Literature". Gastroenterology Research and Practice. 2013: 1–10. doi:10.1155/2013/478349. ISSN 1687-6121.
  7. ↑ 7.0 7.1 Dutly, F.; Altwegg, M. (2001). "Whipple's Disease and "Tropheryma whippelii"". Clinical Microbiology Reviews. 14 (3): 561–583. doi:10.1128/CMR.14.3.561-583.2001. ISSN 0893-8512.
  8. ↑ 8.0 8.1 Dolmans, Ruben A. V.; Boel, C. H. Edwin; Lacle, Miangela M.; Kusters, Johannes G. (2017). "Clinical Manifestations, Treatment, and Diagnosis of Tropheryma whipplei Infections". Clinical Microbiology Reviews. 30 (2): 529–555. doi:10.1128/CMR.00033-16. ISSN 0893-8512.
  9. ↑ 9.0 9.1 Conly JM, Johnston BL (2001). "Rare but not so rare: The evolving spectrum of Whipple's disease". Can J Infect Dis. 12 (3): 133–5. PMC 2094813. PMID 18159328.
  10. ↑ 10.0 10.1 Fenollar, Florence; Lagier, Jean-Christophe; Raoult, Didier (2014). "Tropheryma whipplei and Whipple's disease". Journal of Infection. 69 (2): 103–112. doi:10.1016/j.jinf.2014.05.008. ISSN 0163-4453.
  11. ↑ 11.0 11.1 Keita, Alpha Kabinet; Brouqui, Philippe; Badiaga, Sékéné; Benkouiten, Samir; Ratmanov, Pavel; Raoult, Didier; Fenollar, Florence (2013). "Tropheryma whipplei prevalence strongly suggests human transmission in homeless shelters". International Journal of Infectious Diseases. 17 (1): e67–e68. doi:10.1016/j.ijid.2012.05.1033. ISSN 1201-9712.
  12. ↑ 12.0 12.1 Schwartzman, Sergio; Schwartzman, Monica (2013). "Whipple's Disease". Rheumatic Disease Clinics of North America. 39 (2): 313–321. doi:10.1016/j.rdc.2013.03.005. ISSN 0889-857X.

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