HYPOTHYROIDISM AND PRIMARY PULMONARY-HYPERTENSION - AN AUTOIMMUNE PATHOGENETIC LINK

Primary pulmonary hypertension (PPH), an often fatal disease of unknown cause that primarily affects young women, is a diagnosis of exclusion. The relative roles of genetic predisposition, autoimmunity, viral infection, hormonal influences, and environmental and drug exposures am not known. Among seven patients enrolled by our center in a multicenter trial of continuous prostacyclin in severe PPH, four of the five women had hypothyroidism. Although a causal relationship cannot be established based on this small case series, the possibility of an autoimmune pathogenetic link between PPH and hypothyroidism should be investigated further.