Whipple's disease history and symptoms: Difference between revisions

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


==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 [[arthropathy]]. 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==
 
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]].
== Common Symptoms ==
== Common Symptoms ==
==== Non specific symptoms ====
* [[Fever]]
* [[Weight loss]]
* [[Fatigue]]


====Gastrointestinal system====
* [[Diarrhea]]: The most common complaint
* [[Steatorrhea]]
* [[Abdominal pain]]
* Occult [[mucosal bleeding]]
* [[Ascites]]
* [[Hepatosplenomegaly]]
* [[Hepatitis]]


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


== Less Common Symptoms ==
== Less Common Symptoms ==
====Central nervous system====
* Asymptomatic involvement with detection of [[Tropheryma whipplei]] [[DNA]] in [[CSF]]: The most common finding
* Focal CNS involvement:
** [[Dementia]]
** [[Cerebral ataxia]]
** [[Hemiparesis]]
** [[Personality changes]]
** [[Opthalmoplegia]]
** [[Nystagmus]]
** Supranuclear opthalmoplegia (50% of patients)
** [[Myoclonus]] (25% of patients)
* [[Hypothalamic]] involvement (rarely)
** [[Polydipsia]]
** [[Hyperphagia]]
** Change in [[libido]]
** Change in sleep wake cycle
* [[Eye movement]] abnormalities (pathognomic)
** Oculomasticatory myorhythmia (OMM)
** Oculofacial-skeletal myorhythmia (OSFM)
* CNS involvement usually has poor prognosis.<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="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>
====Ocular involvement====


* [[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. <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="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>
rare and can involve: <ref name="pmid11432814" />
 
* [[uveitis]],
* 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. <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>
* [[retinitis]] ,
* Skeletal muscle involvement in Whipples disease is seen as [[myalgias]] and muscle cramps <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>
* [[vitritis]]  
* 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 preceded by [[arthritis]] and [[arthralgias]] .<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="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>
* [[retrobulbar neuritis]]
* 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. <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>
* [[papilledema]] .  
* Hematological finding in Whipple's disease is [[anemia]] which may be due to malabsorption or mucointestinal bleeding.<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>
* Ocular involvement is often seen in patients with CNS disease. <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>
* 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. <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>
'''Skeletal muscle''' '''involvement'''
* Late in course renal involvement may be seen rarely. <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>
* seen as [[myalgias]] and muscle cramps <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>
'''Cardiac involvement''' :may have varied presentation .<ref name="pmid17202456" /> <ref name="pmid11432814" />
* [[pericarditis]]
* [[myocarditis]]
* [[heart failure]]  
* [[sudden Cardiac death]]  
* [[Endocarditis]] :  associated with negative blood cultures. Endocarditis is often preceded by [[arthritis]] and [[arthralgias]] .<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="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>
'''Pulmonary involvement <ref name="pmid11432814" />'''
* manifest as pleuritic chest pain
* non productive cough
* [[dyspnea]]  
* Chest x-ray often shows [[pleural effusion]] , pulmonary infiltration, or granulomatous mediastinal adenopathy. Often abdominal lymph nodes may be involved.
'''Hematological finding'''
* [[anemia]] which may be due to malabsorption or mucointestinal bleeding.<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>
'''Cutaneous finding'''
* [[melanoderma]] : less frequently reported these days as Whipple's disease is increasingly recognized early. <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>
'''Renal involvemen'''t : late in course, may be seen rarely. <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>


==References==
==References==

Revision as of 20:28, 9 November 2017

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

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.

Common Symptoms

Non specific symptoms

Gastrointestinal system

Articular system

Less Common Symptoms

Central nervous system

Ocular involvement

rare and can involve: [2]

Skeletal muscle involvement

Cardiac involvement :may have varied presentation .[1] [2]

Pulmonary involvement [2]

  • manifest as pleuritic chest pain
  • non productive cough
  • dyspnea
  • Chest x-ray often shows pleural effusion , pulmonary infiltration, or granulomatous mediastinal adenopathy. Often abdominal lymph nodes may be involved.

Hematological finding

  • anemia which may be due to malabsorption or mucointestinal bleeding.[2]

Cutaneous finding

  • melanoderma : less frequently reported these days as Whipple's disease is increasingly recognized early. [1]

Renal involvement : late in course, may be seen rarely. [1]

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 1.5 Fenollar F, Puéchal X, Raoult D (2007). "Whipple's disease". N Engl J Med. 356 (1): 55–66. doi:10.1056/NEJMra062477. PMID 17202456.
  2. ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 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.

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