Severe Precapillary Pulmonary Hypertension Due to Whipple Disease

Background: Whipple disease (WD), a rare systemic infection caused by Tropheryma whipplei, represents an uncommon but recognized cause of reversible pulmonary hypertension (PH). Case summary: A 41-year-old woman with a history of arthritis, eczema, and low-grade fever was referred to our center with severe precapillary PH. Initial diagnostic work-up suggested sarcoidosis, prompting initiation of immunosuppressive therapy. Shortly thereafter, the patient developed severe diarrhea. Subsequent colonoscopy and intestinal biopsy revealed WD. After the initiation of targeted antibiotic therapy, the patient experienced rapid clinical improvement, with complete resolution of symptoms and normalization of hemodynamic parameters. Discussion: This case demonstrates that systemic inflammation in WD can involve the pulmonary vasculature and lead to precapillary PH, which may be fully reversible with timely diagnosis and targeted antibiotic therapy. Take-home message: PH is a pathophysiological condition defined by elevated mean pulmonary arterial pressure arising from primary pulmonary vascular disorders or as a consequence of other conditions such as WD.