Jornal de Pneumologia
Print version ISSN 0102-3586
On-line version ISSN 1678-4642
SOUZA, Eduardo José do Rosário e; GARIB, Junia Rios; GARIB, Nasim Michel and PADUA, Paulo Madureira de. Sildenafil for treatment of pulmonary hypertension in association with systemic lupus erythematosus and anti-phospholipid syndrome. J. Pneumologia [online]. 2003, vol.29, n.5, pp.302-304. ISSN 0102-3586. http://dx.doi.org/10.1590/S0102-35862003000500009.
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.
Keywords : Vasodilator agents [therapeutic use]; Lupus erythematosuos systemic; Antiphospholipid antibodies; Phosphodiesterase inhibitors.