IDIOPATHIC PULMONARY HILAR FIBROSIS - AN UNUSUAL CAUSE OF PULMONARY-HYPERTENSION

A 37-year-old man with progressive exertional dyspnea had pulmonary hy pertension associated with pulmonary arterial and venous obstruction. An autopsy revealed that the cause of death was idiopathic pulmonary h ilar fibrosis, a variant of mediastinal fibrosis. Pulmonary hilar fibr osis can mimic thromboembolic pulmonary hypertension, pulmonary veno-o cclusive disease, and pulmonary venous hypertension.