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

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.

Whipple`s disease - clinical spectrum and diagnostic approach (with a contribution of eight cases)

“…The epidemiological investigations based on a comparative analysis for the last decades of the XX century demonstrate a significant increase of the relative share of the females affected by the disease (2). A chronic asymptomatic carriership was proved through examination of the saliva and fecal samples from healthy individuals (3,7,13). A feco-oral, orooral and air-drop infection transmission route was suggested (8,9).…”

“…WD is rare. Until present days, there are about 1000 case reports of this disease (2,3,7,12,14). Most commonly, Caucasian males at an average age of 50 years are affected.…”

Uma causa pouco habitual de diarreia

“…A DW é uma doença infecciosa sistémica rara, de carácter crónico, causado por um bacilo gram-positivo, o Tropheryma whipplei (TW) pertencente à ordem das Actinomycetales, geralmente encontradas no solo 3,4 . Desde 1980, estima-se que a sua incidência anual ronde os 30 casos por ano 4,5 .…”

“…Desde 1980, estima-se que a sua incidência anual ronde os 30 casos por ano 4,5 . Aproximadamente 80% dos indivíduos infectados são do género masculino, a maioria de raça caucasiana.…”

“…A idade média na altura do diagnóstico é de 50 anos, podendo ocorrer em qualquer faixa etária. A hipótese de contagio por via oral é suportado pela identificação do DNA do TW nas fezes de indivíduos saudáveis e amostras de águas residuais 3,4 .…”