HYPOTHYROIDISM AND PRIMARY PULMONARY-HYPERTENSION - AN AUTOIMMUNE PATHOGENETIC LINK

Primary pulmonary hypertension (PPH), an often fatal disease of unknow n cause that primarily affects young women, is a diagnosis of exclusio n. The relative roles of genetic predisposition, autoimmunity, viral i nfection, hormonal influences, and environmental and drug exposures am not known. Among seven patients enrolled by our center in a multicent er trial of continuous prostacyclin in severe PPH, four of the five wo men had hypothyroidism. Although a causal relationship cannot be estab lished based on this small case series, the possibility of an autoimmu ne pathogenetic link between PPH and hypothyroidism should be investig ated further.