Whipple's disease: multiple hospital admissions of a man with diarrhoea, fever and arthralgia.
Whipple's disease is a rare chronic multi-systemic infectious disorder caused by the Gram-positive bacillus, Tropheryma whippelii. Infection may involve any organ in the body, and most commonly affects white men in the fourth to sixth decades of life. The most common presenting symptoms are gastrointestinal and include abdominal pain, diarrhoea, anorexia and associated weight loss. However, the variability in presentation is considerable and some patients may present with intermittent low-grade fever, neurological abnormalities (nystagmus, ophthalmoplegia, cranial nerve defects), migratory arthralgia, lymphadenopathy, or involvement of the cardiovascular system. In typical Whipple's disease, the most severe changes are seen in the proximal small intestine and biopsy reveals mucosal and lymph node infiltration with large, foamy histocytes, containing granules that stain positive with periodic acid-Schiff (PAS) reagent and represent intact or partially degraded bacteria. Extended antibiotic treatment (up to 1-year) is indicated. Life-long surveillance for recurrence is essential, once primary treatment has been completed. We report the case of a 58-year-old man who developed a rare infection with the actinobacterium, T. whippelii. The patient had suffered intermittent episodes of varying clinical symptoms associated with multiple hospital admissions and clinical diagnoses, spanning a period of 22 years. Historically, arthralgia was the primary manifestation in this patient and also was the chief complaint for which he was first hospitalized 22 years ago. At his most recent admission to our hospital department, his presenting symptoms were persistent fatigue, weight loss, arthralgia and diarrhoea. Thus, it is essential that clinicians retain a high index of suspicion for T. whippelii infection in patients who have a long-term history of arthritis, fever and diarrhoea.
Magira
Eleni E
EE
1st Department of Internal Medicine, Evangelismos General Hospital, 10676 Athens, Greece. elmagira@yahoo.com
Gounaris
Theodore
T
Sioula
Evangelia
E
D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D018771:Arthralgia; D002443:Ceftriaxone / Q000627:therapeutic use; D003937:Diagnosis, Differential; D003967:Diarrhea; D004318:Doxycycline / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004384:Duodenoscopy; D004386:Duodenum / Q000473:pathology*; D005334:Fever; D006801:Humans; D007413:Intestinal Mucosa / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010503:Periodic Acid-Schiff Reaction; D016896:Treatment Outcome; D015431:Weight Loss; D008061:Whipple Disease / Q000175:diagnosis* / Q000188:drug therapy*
D000900:Anti-Bacterial Agents; D002443:Ceftriaxone; D004318:Doxycycline