Abstract
Whipple's disease (WD) is a rare chronic infectious multisystem disease caused by bacterium Tropheryma whipplei. Only about 1000 cases have been reported to date. We describe our experience with 4 cases diagnosed with WD between 2002 and 2007: first 3 cases from Brooklyn, New York and case #4 from Western Massachusetts. Case #1: A 72 year-old (yo) Caucasian male from Spain, living in Brooklyn since 1975, presented with 30 lbs weight loss, pulmonary nodules and retroperitoneal lymphadenopathy. Lymph node biopsy revealed foamy macrophages (FM) containing periodic acid-Schiff (PAS) positive (+) material and negative Ziehl-Neelsen stain (ZN). Retrospectively, he had arthralgia for 10 years and developed intermittent diarrhea, epigastric pain and progressive cognitive decline for the last 6 months. Laboratory data were significant for anemia and evidence of malabsorbtion. On upper endoscopy (EGD) duodenal mucosa had small lymphangiectasia and biopsy was consistent with WD with PAS + FM in lamina propria (LP). Case # 2: A 61 yo Caucasian male from Russia, living in Brooklyn for the last 7 years, with long history of arthralgia was evaluated for iron deficiency anemia. Colonoscopy was unrevealing and EGD showed normal appearing duodenal mucosa with distended villi by FM PAS + and negative ZN on biopsies, consistent with WD. Case #3: A 25 yo African American male presented with diarrhea for 1 year. Colonoscopy with ileoscopy revealed erythema of the terminal ileum mucosa with PAS + FM on biopsies. EGD confirmed the diagnosis of WD with similar findings on duodenal biopsy. Case #4: A 41 yo Caucasian male of Irish descend, with history of seronegative polyarthritis on treatment with high doses of aspirin and intermittent steroids presented with an episode of diarrhea for 4 weeks with negative work-up including colonoscopy. The episode resolved, but subsequently the patient had recurrence of diarrhea and weight loss. On EGD duodenal mucosa appeared flat, pale and biopsies showed PAS + FM in LP suggestive of WD. The diagnosis was confirmed by a PCR assay. All patients were treated with Ceftriaxone for 2 weeks followed by Trimethoprim -sulfamethoxazole for 1 year. Diarrhea and anemia resolved and arthralgia was significant improved in all patients. Patient in case #1 had weight gain and improvement in adenopathy and cognitive function. Discussion: WD has a variety of clinical manifestations and should be considered in patients who present with migratory arthralgias of the large joints, weight loss, diarrhea, and abdominal pain.
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