Whipple’s disease - Disorders of the small bowel and appendix

Whipple’s disease

Definition

Rare multisystem disorder with malabsorption, lymphadenopathy and arthritis.

Aetiology

Caused by an infection by Tropheryma whippelii, an acti-nomycete. The route of infection is unknown. The organism is found in intestinal macrophages.

Clinical features

Patients present with steatorrhoea, abdominal pain and systemic symptoms of fever, weight loss, lymphadenopathy and arthritis. Heart, lung and CNS involvement may occur.

Investigations and management

Electron microscopy can demonstrate the organism. PCR is now available for diagnosis. Various antibiotics are used often in combination and for prolonged courses (up to 1 year).