Primary pulmonary arterial hypertension presenting as diffuse micronodules on CT.

Author: Horton MR, Tuder RM

Author affiliation: Johns Hopkins University School of Medicine, Division of Pulmonary and Critical Care Medicine, Department of Pathology, Baltimore, Maryland 21205, USA. mhorton2@jhmi.edu

Publication date & source: 2004, Crit Rev Comput Tomogr., 45(5-6):335-41.

Publication type: Case Reports

Primary pulmonary arterial hypertension is a rare lethal disease that typically presents radiographically with enlarged central pulmonary arteries, pruning of the peripheral vasculature, and cardiomegaly but clear lung fields. Although it is a disease of unknown etiology, primary PAH has been associated with anorexigen use. We present a case of pulmonary arterial hypertension in a woman with a history of fenfluramine and phentermine use who presented with diffuse micronodules on computed tomography scan. Lung biopsy confirmed the micronodules were radiographic manifestations of extensive diffuse plexogenic arterial lesions. This report represents an unusual radiographic presentation of anorexigen related pulmonary arterial hypertension, and to our knowledge, the first case reported as presenting with diffuse micronodules on high resolution computed tomography scan.