Background: Pulmonary hypertension is an uncommon feature of Whipple's disease and the underlying pathophysiological mechanism remains a subject of debate.
Case report: A 57-year-old woman was hospitalized for exploration of migrating joint pain that had developed for 5 years. Histologically proven Whipple's disease was diagnosed on duodenal biopsies. The lung angiogram performed to explore signs of right heart failure demonstrated pulmonary hypertension and ruled out pulmonary embolism. Abundant pericardial effusion developed progressively. Antibiotic therapy using sulfamethoxazole-trimethoprime led to a systemic Jarisch Herxheilmer reaction. The pulmonary hypertension resolved rapidly, the pericardial effusion more slowly.
Discussion: The pulmonary hypertension in this patient appeared to be directly related to Whipple's disease, probably via vascular infiltration by Tropheryma whippeli.