A 45-year-old man comes to the office due to a 1 year history of abdominal pain and greasy stools. He has also noticed a 7-kg (15.4-lb) weight loss over the past 6 months. Medical history is significant for several years of joint pain in his knees and wrists but is otherwise unremarkable. The patient does not use tobacco or alcohol. After an initial workup, a duodenal biopsy is performed. Microscopic examination shows numerous macrophages in the lamina propria filled with inclusions positive for periodic acid-Schiff stain. Which of the following is the most appropriate treatment for this patient?

Question

A 45-year-old man comes to the office due to a 1 year history of abdominal pain and greasy stools.  He has also noticed a 7-kg (15.4-lb) weight loss over the past 6 months.  Medical history is significant for several years of joint pain in his knees and wrists but is otherwise unremarkable.  The patient does not use tobacco or alcohol.  After an initial workup, a duodenal biopsy is performed.  Microscopic examination shows numerous macrophages in the lamina propria filled with inclusions positive for periodic acid-Schiff stain.  Which of the following is the most appropriate treatment for this patient?

Quizplus · Accepted Answer

This patient with joint pain and malabsorption (eg, greasy stools, weight loss) has biopsy findings consistent with Whipple disease, a rare, systemic disease caused by chronic infection with the gram-positive bacillus Tropheryma whipplei. It usually presents in middle-aged men (age 40-60) and often affects multiple organ systems, most commonly the small intestine (eg, abdominal pain, diarrhea, steatorrhea, weight loss), joints (eg, arthralgia), and CNS (eg, dementia). T whipplei proliferates within the macrophages of affected tissues and provokes minimal inflammatory response, allowing persistent infection. Diagnosis can be confirmed with biopsy of the small intestine, which shows numerous foamy macrophages distended by bacilli in the lamina propria. The bacilli are characteristically positive for periodic acid-Schiff (PAS), which stains polysaccharides in the T whipplei membrane. Long-term (ie, ≥12 months) antibiotic therapy is usually successful in resolving the symptoms, although relapses can occur. (Choice A) Aminosalicylic acid derivatives are appropriate treatment for ulcerative colitis (UC). Although UC can present with gastrointestinal symptoms, weight loss, and joint pain, microscopic examination of the intestine typically shows diffuse mucosal inflammation, including crypt abscesses and ulceration. (Choice C) Digestive enzyme supplementation is appropriate treatment for patients with diminished exocrine pancreatic function (eg, chronic pancreatitis). Patients with chronic pancreatitis may have abdominal discomfort, greasy stools, and weight loss. However, PAS-positive macrophages would not be expected. (Choice D) A modified diet is indicated for patients with celiac disease or other food-sensitive conditions. Celiac disease can present with abdominal discomfort, greasy stools, and weight loss, but histologic findings typically include duodenal villous blunting, increased intraepithelial lymphocytes, and crypt hyperplasia. (Choice E) Surgery is appropriate for patients with bowel conditions unresponsive to medical treatment (eg, ischemic infarction). Intestinal infarction typically presents with severe abdominal pain, and microscopic examination may show coagulative necrosis. Educational objective: Whipple disease, caused by the gram-positive actinomycete Tropheryma whipplei, is a rare systemic illness that often involves the small intestine, joints, and CNS. Classic intestinal histologic findings include foamy macrophages in the lamina propria filled with bacilli positive for periodic acid-Schiff stain. Treatment is with antibiotics. __________ References: Clinical manifestations, treatment, and diagnosis of Tropheryma whipplei infections (http://www.ncbi.nlm.nih.gov/pubmed/28298472)

A 45-Year-Old Man Comes to the Office Due to a 1