Abstract Introduction Whipple’s disease, caused by Tropheryma whipplei, is a chronic systemic infection. Pulmonary hypertension (PH) is an exceptionally rare manifestation, classified under World Health Organization (WHO) Group 5 due to mixed/multifactorial mechanisms. Recognition of this reversible infectious cause of PH is important, as antimicrobial therapy may restore normal pulmonary vascular physiology. Case Presentation A 62-year-old woman with rheumatoid arthritis on adalimumab, atrial fibrillation, supraventricular tachycardia, and type 2 diabetes presented with one month of abdominal pain, diarrhea, and worsening exertional dyspnea. Admission echocardiography showed new severe right ventricle (RV) dilation with moderately reduced systolic function (tricuspid regurgitation velocity 3.2 m/s, tricuspid annular plane systolic excursion 1.5 cm, RV systolic pressure 49 mmHg) compared to a previously normal study four months prior. Computed tomography angiography demonstrated marked RV enlargement, reflux of contrast into the superior vena cava, and a dilated main pulmonary artery without pulmonary embolism. Right heart catheterization confirmed severe precapillary PH (mean pulmonary artery pressure 33 mmHg, pulmonary capillary wedge pressure 10 mmHg, pulmonary vascular resistance (PVR) 5.17 Wood Units) with preserved cardiac output. Abdominal imaging revealed diffuse mesenteric lymphadenopathy. Upper endoscopy with duodenal biopsies demonstrated villous blunting with periodic acid-Schiff-positive macrophages, consistent with Whipple’s disease. Intravenous ceftriaxone was initiated, and she demonstrated marked clinical improvement within one week. She completed two weeks of ceftriaxone, then transitioned to oral doxycycline and hydroxychloroquine. Repeat echocardiography subsequently demonstrated normalization of RV size and function, and repeat right heart catheterization confirmed normalization of filling pressures and PVR. Discussion Whipple’s disease is a rare but clinically relevant cause of reversible precapillary PH. The abrupt onset of RV failure, elevated PVR with normal wedge pressure, and rapid normalization after targeted antimicrobial therapy support an infection-mediated pulmonary vasculopathy rather than fixed vascular remodeling. Pulmonary involvement in Whipple’s disease may precede or overshadow gastrointestinal manifestations and may be more likely to present in immunosuppressed patients, particularly in those receiving anti-tumor necrosis factor therapy This abstract is funded by: None
Allen et al. (Fri,) studied this question.