Last updated: November 4, 2014
Synonyms: Intestinal lipodystrophy.
ICD-9 Code: 040.2.
ICD-10 Code: K90.81
Definition: Whipple’s disease is a multisystem infectious disorder characterized by inflammatory polyarthritis and small bowel colitis.
Etiology: Whipple’s disease is caused by infection with the gram-positive actinomycete Trophermyma whippelii. Intestinal or lymph node biopsy specimens may disclose periodic acid–Schiff staining deposits in macrophages, and electron microscopy may show rod-shaped bacilli.
Demographics: Whipple’s disease is most common in men (90%) older than 40 years of age.
Cardinal Findings: Diarrhea, steatorrhea, weight loss, fever, arthritis, serositis, and lymphadenopathy are seen. Arthralgia or arthritis frequently precedes the intestinal features. The inflammatory arthritis tends to be polyarticular, symmetric, and seronegative and may be chronic or transient.
Uncommon Findings: Iritis, vitreitis, ocular palsy, and progressive encephalitis have been described.
Diagnostic Tests: T. whippelii may be identified by PCR.
Keys to Diagnosis: A high index of suspicion is needed, especially in older men with seronegative RA-like polyarthritis, systemic features, and intestinal disease. Perform small bowel biopsy and a PCR test for the causative agent.
Therapy: Oral trimethoprim-sulfamethoxazole twice daily (or tetracycline 1 g/day) for 1 year is recommended.
Fenollar F, Raoult D. Whipple’s disease. Curr Gastroenterol Rep 2003;5:379–385. PMID:12959718