A Clinician's Pearls and Myths in Rheumatology: Second Edition

Document Type

Book Chapter

Publication Date



Whipple’s disease is a rare, multisystem disorder caused by the bacterium Tropheryma whipplei. Typically presents with intermittent arthritis/arthralgia combined with chronic diarrhea, abdominal pain, and weight loss. Central nervous system and cardiovascular system involvement may also cause symptoms. Asymptomatic carriage of Tropheryma whipplei is common, as the organism is ubiquitous in the environment. It is theorized that this bacterium becomes symptomatic through tissue infiltration in hosts with impaired immunity. Whipple’s disease may be subclassified into classic Whipple’s disease, localized Whipple’s disease, and isolated neurological Whipple’s disease. Patients with gastrointestinal manifestations are most commonly diagnosed by duodenal biopsies showing periodic acid Schiff (PAS) staining of bacteria in macrophages. If untreated, Whipple’s disease can be progressive and even fatal. Successful treatment can be achieved in most cases by antimicrobial therapy. However, Whipple’s disease can relapse because of either persistent or recurrent infection. Immune reconstitution inflammatory syndrome (IRIS) may complicate the course of treatment.

First Page


Last Page


Chapter Title

Whipple's Disease


Springer, Cham