Whipple's disease

Whipple's disease is a rare bacterial infection that causes weight loss, abdominal pain, diarrhea, and arthritis.

CASES/YEAR
15 (US); 300 (Global)
CATEGORY
AGENT TYPE
Bacteria
OTHER NAMES
Intestinal lipodystrophy; Tropheryma whippelii infection;
ACUITY
Subacute/Chronic
INCUBATION
INITIAL SYMPTOMS
Weight loss, abdominal pain, diarrhea, and arthritis; [Merck Manual, p. 153]
PRECAUTIONS
COMMENTS
Whipple's disease (WD) should be suspected in middle-aged white males who have arthritis, abdominal pain, diarrhea, weight loss, and other symptoms of malabsorption; the first symptoms are usually arthritis and fever. [Merck Manual, p. 153] Arthritis is the characteristic symptom that typically precedes other symptoms. [Harrisons, p. 502] Common presentations of WD are chronic GI illness, CNS or eye problems, lymphadenopathy, and fever of unknown origin. Differential diagnoses include Crohn's disease, lymphoma, celiac disease, Still's disease, amyloidosis, and atypical mycobacteria infection. Four different ways to diagnose are: 1.) duodenal biopsy (PAS staining); 2.) PAS staining of macrophages in lymph nodes, brain, and other tissues; 3.) PCR of fluid or tissue; 4.) Immunostains for histopathology (not widely available); Organisms can be found in saliva, subgingival plaques, intestinal biopsies, and stool of healthy individuals. [ABX Guide] The mean age at diagnosis is 50 years, and the disease is 8 times more common in men. Recent data suggest that the intestinal disease is secondary to medical immunosuppression for arthritis. In a large series, 63% of patients had arthropathy, often with HLA-B27 positivity, that preceded the diagnosis by a mean of 8 years. The infection of the intestine disturbs the villous architecture, and on gross inspection, the duodenum and jejunum are thickened and edematous. [PPID, p. 2578-84] Other findings are cognitive change, meningitis, pericarditis, myocarditis, pulmonary infiltration, mediastinal adenopathy, anemia, thrombocytosis, and leukocytosis. [Cohen, p. 186t, 346t] Incidence is higher in farmers. Organisms are detected in individuals without disease--could be carrier state or non-pathogenic types. [Cecil, p. 895]
DIAGNOSTIC
Small bowel biopsy with PAS positive inclusions; Confirm with 2 PCR tests or immunohistology tests; [PPID, 2582-3] "PCR of urine, CSF, blood is decreasing need to obtain brain or other organ tissue for PAS staining." [ABX Guide]
SCOPE
Global
SIGNS & SYMPTOMS
  • >arthralgia
  • >fever
  • >myalgia
  • G abdominal mass
  • G abdominal pain
  • G blood in stool
  • G diarrhea
  • G hepatomegaly
  • H anemia
  • H leukocytosis
  • H lymphadenopathy
  • H splenomegaly
  • N headache
  • N muscle weakness
  • N seizure
  • R chest pain
  • R cough
  • X hilar lymphadenopathy
  • X pleural effusions
  • *arthritis
  • *cranial neuropathy
  • *endocarditis
  • *meningitis
  • *myocarditis
  • *paralysis
  • *pericarditis
  • *uveitis
  • *weight loss
ANTIMICROBIC

Yes

VACCINE

No

ENTRY
RESERVOIR
RISK FACTORS
REFERENCES FOR CASES/YEAR
1. (US) About 30 case per year reported in Western Europe and North America;; [Gorbach, p. 406]
2. (Global) Assume 15 per year in US, then global cases/yr = 20 X 15 = 300