Primary Antiphospholipid Syndrome and Pulmonary Hypertension with Prolonged Survival

Abstract

The outcome of patients with pulmonary hypertension (PHT) and antiphospholipid syndrome (APS) is usually fatal. The authors report the rare case of a patient with primary APS and nonthrombotic PHT who has survived for twenty years after the onset of PHT. In this case, the patient's PHT resembled the primary idiopathic variety with clear lung fields and normal perfusion on the lung scan, and the combination therapy with nitrate, digoxin, and diuretics had been performed. During her clinical course over twenty years, she had not experienced any critical pulmonary thrombosis that influenced the progression of nonthrombotic PHT or any other severe systemic involvement of APS.