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Whipple Disease — SCE Rheumatology MCQ

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HardConnective Tissue DiseaseWhipple DiseaseSCE Rheumatology

A 55-year-old man has a 6-year history of episodic, migratory, non-erosive synovitis affecting the large joints. Rheumatoid factor and anti-CCP antibodies are negative. He now develops chronic diarrhoea, a 9 kg weight loss and diffuse hyperpigmentation. Duodenal biopsy shows expansion of the villous lamina propria by foamy macrophages containing coarse PAS-positive, diastase-resistant granules. Ziehl–Neelsen and Grocott stains are negative. Which diagnosis best explains the complete presentation?

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Correct answer: A — Whipple disease

The diagnosis is Whipple disease, a multisystem infection caused by Tropheryma whipplei. The discriminating pattern is relapsing, migratory seronegative arthritis preceding malabsorptive gastrointestinal disease by several years, together with PAS-positive, diastase-resistant foamy macrophages in the duodenal lamina propria. Tissue T. whipplei PCR or immunohistochemistry should provide organism-specific confirmation because PAS staining is not completely specific and stool or saliva PCR can reflect carriage. Mycobacterium avium complex also produces foamy macrophages, but the organisms are acid-fast. Histoplasma organisms are highlighted by Grocott staining. Coeliac disease causes villous atrophy, crypt hyperplasia and intraepithelial lymphocytosis rather than macrophage accumulation. Amyloidosis produces Congo-red-positive extracellular deposits with apple-green birefringence.

Reference: Cappellini A, Minerba P, Maimaris S, Biagi F. Whipple's disease: A rare disease that can be spotted by many doctors. European Journal of Internal Medicine. 2024;121:25-29. https://pubmed.ncbi.nlm.nih.gov/28298472/