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Jornal de Pneumologia

Print version ISSN 0102-3586

Abstract

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.

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