Sustained success of therapy with inhaled iloprost for severe pulmonary arterial hypertension associated with systemic sclerosis and pulmonary fibrosis.

Case report. We report here the case of a woman with diffuse cutaneous systemic sclerosis with pulmonary involvement and severe (WHO functional class III) pulmonary arterial hypertension (PAH) and recurrent cardiac decompensation. The simultaneous presence of primary biliary cirrhosis with markedly elevated transaminase levels constituted a contraindication for bosentan, which would otherwise have been the first-line treatment for PAH. The patient was therefore treated with inhaled iloprost. Discussion. Once inhaled iloprost therapy had been started, we promptly noted a definite and sustained improvement in physical exercise capacity and normalisation of haemodynamic variables. In cases where bosentan is contraindicated, inhaled iloprost is an effective alternative for the treatment of severe PAH.