PRIMARY ANTIPHOSPHOLIPID SYNDROME AND PULMONARY-HYPERTENSION WITH PROLONGED SURVIVAL - A CASE-REPORT

The outcome of patients with pulmonary hypertension (PHT) and antiphos pholipid syndrome (APS) is usually fatal. The authors report the rare case of a patient with primary APS and nonthrombotic PHT who has survi ved 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 wi th nitrate, digoxin, and diuretics had been performed. During her clin ical course over twenty years, she had not experienced any critical pu lmonary thrombosis that influenced the progression of nonthrombotic PH T or any other severe systemic involvement of APS.