Whipple’s Disease: a Case Report Spanning 20 Years

Abstract

Whipple’s disease is a rare and systemic infectious disease that classically presents with gastrointestinal symptoms. The epidemiology of cardiac involvement is unknown. However, with the advent of improved molecular techniques, it is increasingly recognized as a cause of culture-negative endocarditis. Monotherapy treatment with trimethoprim-sulfamethoxazole is associated with relapses, sometimes in organs different than those previously affected. We present the case of a man diagnosed with classic Whipple disease, initially treated with trimethoprim-sulfamethoxazole. Twenty-two years later, he is diagnosed with endocarditis by the same agent. Whipple’s disease, particularly Whipple’s endocarditis is an extremely difficult entity to diagnose and to treat. Indications for treatment remain controversial and are still based on clinical experience and case series. This particular patient, with a recurrence two decades after the initial episode, highlights the ineffectiveness of trimethoprim/sulfametoxazole for the treatment of Tropheryma whipplei infection, even when used for long periods. Furthermore, this case illustrates the diagnostic difficulty so often associated with endocarditis and the importance of interdisciplinarity when addressing this infection.