Whipple disease is a rare multisystemic disorder of infectious aetiology caused by Tropheryma whipplei. Pulmonary hypertension is a rare association for which the underlying pathophysiological mechanism is unclear. Our patient was a 54-year-old man with a 1-year history of progressive polyarticular arthritis, and worsening respiratory and gastrointestinal symptoms. Pulmonary artery catheterisation demonstrated moderate-to-severe pulmonary hypertension. Duodenal biopsies, with electron microscopy, were diagnostic of Whipple disease. Involvement by Whipple disease was also evident in the stomach, bone marrow and pulmonary pleura. A 2-week course of intravenous ceftriaxone was initiated and this was followed by a 1-year course of trimethoprim/sulfamethoxazole (160/800), once daily. Nine months into antibiotic treatment, a repeat echocardiogram showed normalisation of the size and function of the cardiac chambers, including the right atrium and right ventricle. There was complete resolution of the severe tricuspid insufficiency and pulmonary hypertension. Whipple disease is not generally considered as a possible cause of pulmonary hypertension but such awareness is important given that it may be potentially reversible with antibiotic therapy.
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Competing interests: none.
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