Tropheryma whipplei
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Tropheryma whipplei /
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Background
Microbiology
- Fastidious Gram-positive bacillus
Epidemiology
- Ubiquitous environmental organism, but rarely causes disease
- Can be found in saliva of one third of healthy people
- Disease is more common in white European males
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
- 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