Pulmonary fibrosis can severely disrupt lung function, often with fatal consequences. Although the etiology of pulmonary fibrotic diseases is varied, the cause of one pulmonary fibrotic conditions, idiopathic pulmonary fibrosis (IPF), is still unclear. This article will discuss the major pathogenetic factors responsible to cause pulmonary fibrosis.

The prevalence and incidence of pulmonary fibrotic diseases are hard to estimate, given the vast array of clinical conditions. In addition, there are many other fibrotic diseases of the lung including cystic lung disease, scleroderma, radiation and chemotherapy-induced fibrosis, granulomatous lung disease, sarcoidosis and environmental, and smoking-associated COPD. Pathogenesis of pulmonary fibrosis Cystic fibrosis and cystic lung disease With regard to etiology, cystic fibrosis (CF) is unique among pulmonary fibrotic conditions and can be…