Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic condition of unknown aetiology with deteriorating respiratory function leading to respiratory failure. Sequential acute lung injury leads to progressive fixed tissue fibrosis, architectural distortion and loss of function. An excess of profibrotic cytokines and/or a deficiency in antifibrotic cytokines have been implicated in the pathological process as has excessive oxidation. At present no specific therapy is available. 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 prove to be of benefit but data from studies are limited. Novel drugs, mainly antifibrotic, anticytokine and immunoregulatory, are currently being investigated in various trial phases. Most recently, endothelin receptor antagonists (e.g., bosentan) have been shown to have possible beneficial effects in early stages of IPF. After a short overview on the current hypothesis on pathophysiology in IPF this review will discuss the present and possible future therapeutic options in IPF.

Keywords: Acetylcysteine, azathioprine, glucocorticosteroid, idiopathic pulmonary fibrosis, treatment