Introduction: Pulmonary hypertension (PH) is a serious complication of lupus. The effectiveness of current vasoactive therapy has not been well described.
Methods: Retrospective analysis of 12 patients with lupus-associated PH (age 43+/-10 years, mean+/-SD, all female) treated with pulmonary vasodilators.
Results: At baseline, patients had severe PH: median six-minute walk distance (6MWD) 266 m (95% confidence interval [CI], 106 to 362); functional class III (n=7) and IV (n=5); mean pulmonary artery pressure (mPAP) 52 mmHg and cardiac index 2.23 L/min/m(2). Eight patients were started on epoprostenol and 2 each on bosentan or treprostinil. After a mean follow-up of 41+/-25 months, 5 patients were on combination therapy (3 epoprostenol plus bosentan, 1 treprostinil plus bosentan, 1 bosentan plus sildenafil) and 7 were on monotherapy (2 epoprostenol, 4 bosentan, 1 sildenafil); 6MWD increased by 139 m (95% CI, 36 to 259, p=0.007), 8 patients were functional class I or II and 4 were class III; right ventricular systolic pressure (RVSP) decreased by 22 mmHg (95% CI, 6 to 36; p=0.012), mPAP decreased by 18 mmHg (95% CI, 8 to 29; p=0.014), and cardiac index increased by 1.44 L/min/m(2) (95% CI, 0.76 to 2.08; p=0.016). There was no mortality or need for lung transplantation. Therapy was well tolerated.
Conclusions: Vasoactive therapy can achieve sustained clinical and hemodynamic improvement in lupus-associated PH.