Sildenafil no tratamento da hipertens？o pulmonar associada a lúpus eritematoso sistêmico e síndrome antifosfolipídio

severe pulmonary hypertension is a debilitating disease with short life expectancy that often affects young people. pleuropulmonary complications of systemic lupus erythematosus occur in 50-70% of patients. severe symptomatic pulmonary hypertension in systemic lupus erythematosus is rare and carries a bad prognosis because a fatal outcome can occur within months. the authors describe, for the first time, a patient with systemic lupus erythematosus with severe pulmonary hypertension and secondary antiphospholipid syndrome who responded favorably to oral sildenafil, after unsuccessful use of prednisone, intravenous cyclophosphamide, warfarin and diltiazem.