Severe precapillary pulmonary hypertension in suspected antiphospholipid syndrome - partially successful long-term therapy with continuous intravenous iloprost administration

Undifferentiated connective tissue disease with secondary antiphospholipid syndrome was diagnosed in a 33 year old woman after recurrent arterial thro mboses, two miscarriages and myocarditis. Despite effective immunosuppressi on and anticoagulation her initially mild precapillary pulmonary hypertensi on progressed in the absence of thromboembolic events, cardiopulmonary dise ase or other systemic disorders. With continuous i.v. iloprost in a dosage of up to 4.5 ng/kg/min for a total of 15 months, her 6 min walking-distance improved from 210 to 315 m, the pulmonary vascular resistance decreased fr om 1710 to 1111 dynxsxcm(-5) and the mean pulmonary arterial pressure decre ased from 64 to 54 mmHg. This partial success of conservative treatment ena bled a reassessment of the necessity for heart and lung transplantation.