Review Question - QID 212470

This patient’s arthralgia, malabsorptive diarrhea, and characteristic periodic acid-Shiff (PAS)-positive macrophages on histology are characteristic of Whipple disease, which is initially treated with penicillin/ceftriaxone followed by trimethoprim-sulfamethoxazole.

Whipple disease is due to Tropheryma whipplei, a gram-positive, non-acid-fast, PAS-positive rod. The organism is found almost everywhere in the environment and rarely causes chronic disease. It most commonly occurs in older, white males. The disease presents over time with joint symptoms such as migratory arthralgias of large joints. Later in the course, patients will present with intermittent diarrhea with malabsorptive symptoms. Other systems, such as the cardiac or central nervous system, may also be affected. The diagnosis of Whipple disease can be made with the classic finding of PAS-positive macrophages from a small-bowel biopsy. Treatment includes an initial intravenous antibiotic such as penicillin or ceftriaxone followed by maintenance therapy with oral trimethoprim-sulfamethoxazole.

Figure/Illustration A demonstrates PAS-positive macrophages (black arrows) in the lamina propria of the small bowel.

Incorrect Answers:
Answer 1: Avoiding gluten may be beneficial in patients with Celiac disease. Although this patient reports suspected exacerbation of symptoms following bread consumption, his histological finding is more consistent with Whipple disease.

Answer 2: Hydroxychloroquine is used in the treatment of rheumatoid arthritis, which presents with symmetric joint pain with morning stiffness. This patient’s joint symptoms do not follow the classic presentation, and his gastrointestinal symptoms are not explained by rheumatoid arthritis.

Answer 3: Methotrexate is a folate antagonist often used in the treatment of autoimmune diseases such as rheumatoid arthritis and inflammatory bowel disease. This patient’s histological findings confirm the diagnosis of Whipple disease.

Answer 4: NSAIDs are used for a variety of conditions including osteoarthritis, systemic lupus erythematosus, or pain management. This patient’s condition requires antibiotics for treatment.

Bullet Summary:
Whipple disease may present with joint symptoms, malabsorptive diarrhea, and periodic acid-Schiff-positive macrophages; it is best treated penicillin/ceftriaxone followed by trimethoprim-sulfamethoxazole.