Idiopathic pulmonary fibrosis (IPF) is a chronic condition of unknown etiology with deteriorating respiratory
function leading to respiratory failure. Corticosteroids, alone or in combination with immunosuppressive drugs such as
azathioprine, colchicine, and cyclophosphamide, have been used with limited success. Interferon-gamma-1b showed a
significant improvement in pulmonary function only in one study. Pirfenidone, cyclosporine and acetylcysteine may also
be of benefit but data from studies are limited. Novel drugs, mainly antifibrotic, anticytokine and immunoregulatory, are
currently being investigated in various trial phases. Endothelin receptor antagonists have been shown to have possible
beneficial effects in early stages of IPF. However, most recently, the so-called triple combination therapy, anticoagulation
therapy and endothelin receptor antagonists, especially ambrisentan, are either harmful or ineffective in IPF and are not
recommended. We report a brief review on the present and possible future therapeutic options in IPF.
Keywords: Ambrisentan, bosentan, cyclophosphamide, hypertension, idiopatic pulmonary fibrosis, iloprost, pulmonary
pulmonary fibrosis, pirfenidone, sildenafil.
Rights & PermissionsPrintExport