Whipple's disease

Whipple's disease is a rare multi-system disorder caused by Tropheryma whippelii infection. It is more common in those who are HLA-B27 positive and in middle-aged men

Features

• malabsorption: diarrhoea, weight loss
• large-joint arthralgia
• lymphadenopathy
• skin: hyperpigmentation and photosensitivity
• pleurisy, pericarditis
• neurological symptoms (rare): ophthalmoplegia, dementia, seizures, ataxia, myoclonus

Investigation

• jejunal biopsy shows deposition of macrophages containing Periodic acid-Schiff (PAS) granules

Management

• guidelines vary: oral co-trimoxazole for a year is thought to have the lowest relapse rate, sometimes preceded by a course of IV penicillin