Idiopathic pulmonary fibrosis ( IPF) is a group of chronic inflammatory interstitial lung diseases of unknown etiology, characterized by diffuse alveolitis, structural disorders of alveolar units and pulmonary fibrosis, and is one of the most important types of pulmonary fibrotic diseases. Its pathogenesis may be associated with viral infections, oxidative stress, autoimmunity, drugs, inhalation of organic or inorganic dust, smoking, and gastroesophageal reflux disease.IPF is usually disseminated, but it can also occur in families. Familial idiopathic pulmonary fibrosis ( FIPF) has been reported to account for approximately 0.5%-2.2% of total IPF. Several genetic studies carried out in recent years suggest that the presence of genetic factors or congenital susceptibility factors may be associated with the disease. The research progress of pulmonary surface active protein gene, ELMOD2 gene and telomerase gene with FIPF is now highlighted to provide information for further research of FIPF. Wang Haifeng, Department of Pulmonary Diseases, The First Affiliated Hospital of Henan College of Traditional Chinese Medicine