19th July 2023

Whipple’s Disease

Whipple’s disease is a rare disease caused by infection with Tropheryma whipplei – a gram positive actinobacteria. It most commonly affects middle-aged men. Early symptoms include abdominal pain, joint pain, weight loss, diarrhoea, lymphadenopathy, steatorrhoea and skin hyperpigmentation. The disease later spreads and affects the central nervous system (CNS) and heart. CNS symptoms include synchronised abnormal ocular and masticatory movements, abnormal facial movements, memory or consciousness impairment, seizures and ataxia. Cardiac manifestations include endocarditis or myocarditis. Diagnosis is made based on jejunal biopsies taken at upper GI endoscopy (PAS-positive granules in the villi) or by PCR of CSF or lymph nodes (positive for T.Whipplei DNA). Initial treatment of Whipple’s disease is 2 weeks of IV ceftriaxone or benzylpenicillin. This is followed by a 1-year maintenance treatment of oral co-trimoxazole. If there is CNS involvement, oral doxycycline and hydroxychloroquine are added.