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{{Whipple's disease}} | {{Whipple's disease}} | ||
{{CMG}}; {{AE}} {{ | {{CMG}};{{AE}}{{BHA}} | ||
==Overview== | ==Overview== | ||
*Patients with Whipple's disease have varied presentation. | |||
*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== | ||
== | == Common Symptoms == | ||
*[[ | * [[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]] : 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> | |||
== Less Common 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. <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 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> | ||
* 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> | |||
* 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> | |||
* 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> | |||
* [[ | * 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> | ||
* 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> | |||
* 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> | |||
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==References== | ==References== | ||
Revision as of 02:52, 2 November 2017
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Whipple's disease Microchapters |
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Treatment |
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Case Studies |
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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]
Overview
- Patients with Whipple's disease have varied presentation.
- 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
Common Symptoms
- 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. [1]
- 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. [1]
Less Common 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. [1] [2]
- 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. [2]
- Skeletal muscle involvement in Whipples disease is seen as myalgias and muscle cramps [1]
- 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 .[1] [2]
- 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. [2]
- Hematological finding in Whipple's disease is anemia which may be due to malabsorption or mucointestinal bleeding.[2]
- 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. [1]
- Late in course renal involvement may be seen rarely. [1]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Fenollar F, Puéchal X, Raoult D (2007). "Whipple's disease". N Engl J Med. 356 (1): 55–66. doi:10.1056/NEJMra062477. PMID 17202456.
- ↑ 2.0 2.1 2.2 2.3 2.4 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.