Enfermedad de Whipple

Abstract

Whipple's disease (WD) is a multisystemic chronic disease caused by Tropheryma whipplei, a bacterium taxonomically closely related to the group of Actinomyces. Pathogen is transmitted by the oral-faecal route. Bacteria reaches the small intestine, spreads to bloodstream and is disseminated to the different organs and systems. There are different entities of infection: asymptomatic carrier, self-limited WD, classical-digestive WD, localized WD, and others. In classical WD (76% of the cases) the digestive system is usually affected. Diarrhea, abdominal pain and weight loss are the major symptoms, frequently preceded or accompanied by polyarthritis and fever (25-40%). Central nervous system (CNS) involvement is a delayed and advanced form and can manifest as supranuclear ophthalmoplegia or as choreiform movements, hypersomnia, ataxia, seizures or extrapyramidalism, among others. Induction treatment consists in ceftriaxone (2g per day intravenously), along 2 weeks (extendable up to 4 weeks if CNS or cardiac valves impaired). Doxycycline 100 mg/12hours plus hydroxychloroquine 200 mg/8hours at least for 12 months are used in maintenance treatment.