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Whipple’s Disease: Our Own Experience and Review of the Literature

DOI: 10.1155/2013/478349

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Abstract:

Whipple’s disease is a chronic infectious systemic disease caused by the bacterium Tropheryma whipplei. Nondeforming arthritis is frequently an initial complaint. Gastrointestinal and general symptoms include marked diarrhoea (with serious malabsorption), abdominal pain, prominent weight loss, and low-grade fever. Possible neurologic symptoms (up to 20%) might be associated with worse prognosis. Diagnosis is based on the clinical picture and small intestinal histology revealing foamy macrophages containing periodic-acid-Schiff- (PAS-) positive material. Long-term (up to one year) antibiotic therapy provides a favourable outcome in the vast majority of cases. This paper provides review of the literature and an analysis of our 5 patients recorded within a 20-year period at a tertiary gastroenterology centre. Patients were treated using i.v. penicillin G or amoxicillin-clavulanic acid + i.v. gentamicin for two weeks, followed by p.o. doxycycline (100?mg per day) plus p.o. salazopyrine (3?g per day) for 1 year. Full remission was achieved in all our patients. 1. Introduction Whipple’s disease is a rare, chronic, and infectious systemic disease caused by the bacterium Tropheryma whipplei, a member of the diverse order of Actinomycetales, usually found in soil [1]. 2. History In 1907, George Hoyt Whipple described a case of a 36-year-old physician (medical missionary) [2]. Whipple published this report only two years after his graduation from the Johns Hopkins University in 1905. Subsequently he won the Nobel Prize in physiology and medicine (for his “discovery concerning liver therapy of anaemia” in 1934). Whipple described his case as “gradual loss of weight and strength, stools consisting chiefly of neutral fat and fatty acids, indefinite abdominal signs, and a peculiar multiple arthritis” [2]. The patient died of this progressive illness. Whipple called it intestinal lipodystrophy since he observed the accumulation of??“large masses of neutral fats and fatty acids in the lymph spaces.” The illness was renamed Whipple’s disease in 1949 [3]. An infectious aetiology was suspected as early as Whipple’s initial report. Whipple described a “number of rod-shaped organisms resembling in form the tubercle bacillus” in the vacuoles of the foamy cells [2]. Until the early 1960s, the disease was considered to be a uniformly fatal and untreatable primary disorder of fat metabolism. In 1952, the first successful treatment with antibiotics was reported (a prolonged period of chloramphenicol) [4]. Bacillary bodies were identified by transmission electron microscopy,

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