Characterization of Prostacyclin-associated Leg Pain in Patients with Pulmonary Arterial Hypertension

Rationale: Prostacyclin-associated leg pain is a potentially debilitating adverse effect of prostacyclin therapy for patients with pulmonary arterial hypertension (PAH). However, to our knowledge, this entity has not been systematically studied.Objectives: To characterize the clinical features and metabolic risk factors for prostacyclin-associated leg pain.Methods: At one academic medical center, we assembled and analyzed a case series of patients with PAH and prostacyclin-associated leg pain.Measurements and Main Results: Over a period of 2 years, we identified 11 patients with PAH and prostacyclin-associated leg pain who agreed to participate in this study. Subjects underwent a standardized clinical evaluation, electrodiagnostic assessment, and serologic screen for metabolic causes of peripheral neuropathy. All 11 patients were female; their mean (SD) age was 50 (±9) years; their median (interquartile range) PAH duration was 56 (20–96) months; and their prostacyclin therapy duration was a median (interq...