Whipple’s Disease: A Rare Cause of Malabsorption Syndrome

Abstract

Introduction: Whipple's disease is a rare, chronic, systemic disease caused by the actinomycete Tropheryma whipplei. Clinical manifestations vary widely depending on the affected system, the most common being the digestive tract. Case Presentation: The authors report the case of a 52-year-old man with malabsorption syndrome, diarrhea, marked weight loss, melanoderma, and visual and proprioception disorders. Periodic acid-Schiff staining of a proximal small bowel biopsy and peripheral-blood PCR identification of T. whipplei confirmed the disease. The patient was initially treated with intravenous ceftriaxone, followed by oral trimethoprim/sulfamethoxazole with significant clinical improvement. Conclusions: This case is reported due to its rarity and the diagnostic challenge it presents. Although uncommon, Whipple's disease should be considered as a differential diagnosis of malabsorption syndrome due to its systemic impact and possible treatment with targeted antibiotic therapy.sua raridade e pelo desafio diagnóstico. Apesar de incomum deve ser considerada como diagnóstico diferencial do síndrome de má absorção atendendo ao seu impacto sistémico e ao possível tratamento com antibioterapia dirigida.

Whipple’s disease review, prevalence, mortality, and characteristics in the United States: A cross-sectional national inpatient study

“…Historically, case-dependent estimates have placed the prevalence of WD in the general population at around 1 in 1 million. [ 14 ] However, a recent population-based study estimated the US prevalence to be as high as 9.8 in 1 million, [ 15 ] compared to the prevalence in Italy which is 3 in 1 million. [ 16 ]…”

Enfermedad de Whipple: revisión sistemática de la literatura

“…La enfermedad de Whipple (EW) es una enfermedad crónica sistémica con predilección por el aparato digestivo, especialmente el intestino delgado; fue descrita por primera vez en 1907 por George H. Whipple, quien la denominó lipodistrofia intestinal (1,2) . Es causada por una bacteria grampositiva perteneciente a la familia de los Actinomycetaceae denominada Tropheryma whipplei (TW) y la infección generada por esta bacteria presenta síntomas inespecíficos; sin embargo, la presentación clásica comienza con artralgia seguida de diarrea, pérdida de peso, fiebre, adenopatías y, ocasionalmente, manifestaciones neurológicas, cardíacas u oculares; el síndrome febril de origen desconocido es una manifestación atípica de esta enfermedad (1)(2)(3)(4) .…”