Diagnosis and treatment of interstitial lung disease: focusing on idiopathic pulmonary fibrosis

ABSTRACT

Interstitial lung disease (ILD) is a rare condition characterized by extensive inflammation and fibrosis mainly involving the pulmonary interstitium or alveoli. Usually, patients with ILD clinically present with chronic cough and exertional dyspnea. ILD is classified into subtypes based on clinical characteristics, detailed history obtained from patients, and radiological, and/or histopathological features. The most common type of idiopathic interstitial pneumonia is idiopathic pulmonary fibrosis (IPF). IPF is a chronic progressive fibrosing ILD and is associated with poor prognosis. An exclusive diagnosis of IPF requires no known condition causing ILD and typical radiological and/or histopathological features of lung fibrosis. Fibrosis observed in this condition is attributable to repetitive epithelial injury with consequent abnormal wound healing in genetically susceptible and elderly individuals. Currently, pirfenidone and nintedanib are useful disease-modifying agents available to treat IPF. In this article, we review the concept, diagnosis, clinical course, and treatment of ILD.