Whipple’s disease

Abstract

Whipple's disease (WD) is a chronic debilitating disease caused by the bacillus Tropheryma whippleii. WD classically presents with the main clinical symptoms of polyarthralgias, chronic diarrhea, weight loss, and abdominal pain. Given its systemic involvement, it is common for WD to present with a multitude of other clinical scenarios--sometimes with predominant neurologic, cardiac, and dermatologic manifestations. WD can occur at any age, but it generally occurs during the fifth decade and predominantly in men. The diagnosis of WD is established by demonstrating the organism on biopsies from the involved system, by histology, electron microscopy, polymerase chain reaction, and more recently, by culture of bacteria. The histologic features include a coarse granular cytoplasm and foamy macrophages that stain strongly with the period-acid Schiff reagent. Current therapy includes an initial 2-week course of intravenous cephalosporins followed by 1-year oral trimethoprim-sulfamethoxazole.