Idiopathic Pulmonary Fibrosis: Treatment and Prognosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease with a prognosis that can be worse than for many cancers. The initial stages of the condition were thought to mainly involve chronic inflammation; therefore, corticosteroids and other drugs that have anti-inflammatory and immunosuppressive actions were used. However, recently, agents targeting persistent fibrosis resulting from aberrant repair of alveolar epithelial injury have been in the spotlight. There has also been an increase in the number of available antifibrotic treatment options, starting with pirfenidone and nintedanib. These drugs prevent deterioration but do not improve IPF. Therefore, nonpharmacologic approaches such as long-term oxygen therapy, pulmonary rehabilitation, and lung transplantation must be considered as additional treatment modalities.

Keywords: GAP score; clinical trial; idiopathic pulmonary fibrosis; treatment.

Figure 1

Variable clinical course of IPF. Notes: The natural history of idiopathic pulmonary fibrosis (IPF) is highly variable, and the course of the disease in an individual patient is difficult to predict. Some patients experience rapid decline, others progress more slowly, and some patients remain stable. Some patients may experience acute exacerbation of the disease, which might be fatal.