Pulmonary hypertension in systemic Lupus Erythematosus complicated by exposure to Dexfenfluramine

Abstract

Pulmonary arterial hypertension (PAH) is a well recognized, albeit uncommon, complication of systemic lupus erythematosus (SLE) and is characterized histologically by the presence of onionskin thickening of small arterioles. We described a 24 year-old female with SLE, complicated by pulmonary hypertension after a six-week exposure to dexfenfluramine, who died of acute myocardial infarction complicated with pneumococcal sepsis. Autopsy demonstrated pulmonary arteriolar thickening with severe sclerosis as well as the more advanced plexiform and angiomatoid lesions. Based on these findings, we hypothesize that in this case the use of dexfenfluramine, albeit for a relatively short period, exerted a synergistic effect on the predisposition to PAH already inherent in this patient with SLE.