A Rare Case of Pulmonary Hypertension, Misdiagnosed as Interstitial Lung Disease: Pulmonary Capillary Hemangiomatosis.

Abstract

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension. We reported a histologically confirmed PCH in a 42-yr-old lady. She presented a progressive dyspnea and cough after an upper respiratory tract infection. She had a leukocytosis and elevated ESR with negative collagen vascular laboratory results. Her chest imaging revealed mediastinal lymphadenopathy with bilateral ground glass opacities with increased interstitial septal thickening in lung parenchyma. Patient echocardiography showed severe right ventricular dilatation with a measured systolic pulmonary arterial pressure of about 105mmHg. Right heart catheterization revealed a mean pulmonary arterial pressure on 30 mmHg with a pulmonary capillary wedge pressure of about 7 mmHg. After starting anti PH treatment, the patient suffered a pulmonary edema and due to abnormal patient response to anti-PH therapies and radiologic findings. Finally, open lung biopsy was performed and showed features of pulmonary capillary hemangiomatosis.