Primary Pulmonary Hypertension Complicated by Early-Stage Pulmonary Sarcoidosis. A Case Report

A 25 year-old Japanese male was followed up for 2.5 years under a clinical diagnosis of primary pulmonary hypertension and died with intractable right ventricular failure. A postmortem examination revealed intimal thickening, necrotizing arteritis and plexiform lesions of the pulmonary arteries compatible with plexogenic pulmonary arteriopathy. It was also noted that he had clinically unrecognized pulmonary sarcoidosis of early stage, characterized by granulomatous lesions composed of epithelioid cells and Langhans giant cells with little fibrous development. We concluded that this was a case of primary pulmonary hypertension compl icated by pulmonary sarcoidosis, which was not considered to be a cause of the pulmonary hypertension.