Tropheryma whipplei: Difference between revisions
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Tropheryma whipplei
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− | == |
+ | ==Background== |
− | === |
+ | ===Microbiology=== |
− | * |
+ | *Fastidious [[Stain::Gram-positive]] [[Cellular shape::bacillus]] |
− | === |
+ | ===Epidemiology=== |
− | * |
+ | *Ubiquitous environmental organism, but rarely causes disease with only about 12 new cases diagnosed annually worldwide |
− | ** |
+ | **Can be found in saliva of one third of healthy people |
− | * |
+ | *Disease is more common in white European (98%) males (85%) |
+ | *Farming or occupational soil/animal exposures are commone |
||
− | === |
+ | ===Pathophysiology=== |
− | * |
+ | *Lack of host immune response |
− | == |
+ | ==Clinical Manifestations== |
− | === |
+ | ===Classic Whipple's Disease=== |
− | * |
+ | *Cardinal features include: [[arthralgias]], followed by [[weight loss]], [[diarrhea]], and [[abdominal pain]] |
− | * |
+ | *Arthralgias are typically migratory in the larger peripheral joints, including knees, ankles, and wrists, but can have essentially any presentation |
+ | **May be present for years before development of other symptoms |
||
− | * |
+ | *The diarrhea is intermittent, with colicky abdominal pain |
− | ** |
+ | **Diarrhea can be watery or have steatorrhea |
− | ** |
+ | **Occasional GI bleeding |
− | === |
+ | ===Other Symptoms=== |
− | * |
+ | *Fever in 25 to 40% |
− | * |
+ | *Lymphadenopathy, mostly of mesenteric and mediastinal nodes |
− | * |
+ | *CNS disease |
− | ** |
+ | **[[Dementia]], [[supranuclear ophthalmoplegia]], [[nystagmus]], and [[myoclonus]] |
− | ** |
+ | **[[Oculomasticatory myorhythmia]] and [[oculo-facial-skeletal myorhythmia]] with a supranuclear vertical gaze palsy |
− | ** |
+ | **[[Cerebellar ataxia]] |
− | ** |
+ | **Symptoms can occur with disease or as a post-treatment relapse |
− | * |
+ | *Cardiac disease |
− | ** |
+ | **Culture-negative [[endocarditis]], [[pericarditis]], and [[myocarditis]] |
− | ** |
+ | **Endocarditis may occur on its own without other features of disease |
− | * |
+ | *Skin hyperpigmentation in 40% |
− | * |
+ | *[[Pleural effusion]], [[chronic cough]], [[interstitial lung disease]], [[pulmonary hypertension]] |
− | * |
+ | *[[Anemia]] in 80%, [[leukocytosis]] in 50%, [[thrombocytosis]] in 50% |
− | * |
+ | *Elevated C-reactive protein in 70% |
− | == |
+ | ==Diagnosis== |
− | * |
+ | *Samples should be taken from involved sites, with a strong preference for small bowel biopsy if there are GI symptoms, but also synovial or cerebrospinal fluid or heart valve |
− | ** |
+ | **Up to 7 to 10 biopsies from small bowel, ideally |
− | * |
+ | *Diagnosis is based on: |
− | ** |
+ | **Periodic acid-Schiff-positive bacilli in macrophages |
− | ** |
+ | **PCR |
− | ** |
+ | **Immunohistochemistry |
− | == |
+ | ==Management== |
− | * |
+ | *Generally treat with parenteral [[Is treated by::ceftriaxone]] 2 g IV daily or [[Is treated by::penicillin]] 2 MU IV q4h for 2 weeks followed by [[TMP-SMX]] DS PO bid for 1 year |
− | ** |
+ | **Treatment can precipitate a [[Jarisch-Herxheimer reaction]] |
− | ** |
+ | **May also see [[immune reconstitution inflammatory syndrome]] in early treatment |
− | * |
+ | *Other options include [[meropenem]], [[doxycycline]], [[macrolides]], [[ketolides]], [[aminoglycosides]], [[rifampin]], [[teicoplanin]], and [[chloramphenicol]] |
− | * |
+ | *Consider repeating small bowel biopsies annually for a few years |
− | == |
+ | ==Prognosis== |
− | * |
+ | *Clinical improvement takes 1 to 3 weeks of treatment |
− | * |
+ | *Neurologic sequelae may be permanent |
− | * |
+ | *Relapses after treatment, including of CNS disease, can happen in up to a third of patients |
{{DISPLAYTITLE:''Tropheryma whipplei''}} |
{{DISPLAYTITLE:''Tropheryma whipplei''}} |
Revision as of 22:30, 2 August 2020
Background
Microbiology
- Fastidious Gram-positive bacillus
Epidemiology
- Ubiquitous environmental organism, but rarely causes disease with only about 12 new cases diagnosed annually worldwide
- Can be found in saliva of one third of healthy people
- Disease is more common in white European (98%) males (85%)
- Farming or occupational soil/animal exposures are commone
Pathophysiology
- Lack of host immune response
Clinical Manifestations
Classic Whipple's Disease
- Cardinal features include: arthralgias, followed by weight loss, diarrhea, and abdominal pain
- Arthralgias are typically migratory in the larger peripheral joints, including knees, ankles, and wrists, but can have essentially any presentation
- May be present for years before development of other symptoms
- The diarrhea is intermittent, with colicky abdominal pain
- Diarrhea can be watery or have steatorrhea
- Occasional GI bleeding
Other Symptoms
- Fever in 25 to 40%
- Lymphadenopathy, mostly of mesenteric and mediastinal nodes
- CNS disease
- Dementia, supranuclear ophthalmoplegia, nystagmus, and myoclonus
- Oculomasticatory myorhythmia and oculo-facial-skeletal myorhythmia with a supranuclear vertical gaze palsy
- Cerebellar ataxia
- Symptoms can occur with disease or as a post-treatment relapse
- Cardiac disease
- Culture-negative endocarditis, pericarditis, and myocarditis
- Endocarditis may occur on its own without other features of disease
- Skin hyperpigmentation in 40%
- Pleural effusion, chronic cough, interstitial lung disease, pulmonary hypertension
- Anemia in 80%, leukocytosis in 50%, thrombocytosis in 50%
- Elevated C-reactive protein in 70%
Diagnosis
- Samples should be taken from involved sites, with a strong preference for small bowel biopsy if there are GI symptoms, but also synovial or cerebrospinal fluid or heart valve
- Up to 7 to 10 biopsies from small bowel, ideally
- Diagnosis is based on:
- Periodic acid-Schiff-positive bacilli in macrophages
- PCR
- Immunohistochemistry
Management
- Generally treat with parenteral ceftriaxone 2 g IV daily or penicillin 2 MU IV q4h for 2 weeks followed by TMP-SMX DS PO bid for 1 year
- Treatment can precipitate a Jarisch-Herxheimer reaction
- May also see immune reconstitution inflammatory syndrome in early treatment
- Other options include meropenem, doxycycline, macrolides, ketolides, aminoglycosides, rifampin, teicoplanin, and chloramphenicol
- Consider repeating small bowel biopsies annually for a few years
Prognosis
- Clinical improvement takes 1 to 3 weeks of treatment
- Neurologic sequelae may be permanent
- Relapses after treatment, including of CNS disease, can happen in up to a third of patients