Current and future treatment for idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fibrosing interstitial lung disease, which is associated with a short survival rate. The decline in forced vital capacity in patients with IPF appears to be almost the same rate regardless of baseline lung function status. This suggests that early treatment would be necessary to prevent further deterioration even lung function is maintained within normal limits. Both pirfenidone and nintedanib significantly slow the decline in lung function, reduce the risk of acute exacerbation, and improve survival rate. However, many individuals with IPF remain untreated. Most IPF patients can tolerate antifibrotic drug therapy, and the dose adjustment has been shown to effectively reduce side effects without modifying efficacy. Although the recent introduction of pirfenidone and nintedanib has led to the slowing of lung function decline, there is no evidence of fibrosis reversal. In the near future, several new drugs are expected to be prescribed to patients with IPF. We are anticipating that some drugs may reverse fibrosis. Fibrosis inhibiting drugs have different pharmacological actions and there are various mechanisms causing fibrosis in the lesion. Therefore, it is imperative to launch efforts to optimize antifibrotic effects through a combination therapy of several drugs. These efforts will hold out hope for patients with IPF.