Diagnosis and Management of Fibrotic Interstitial Lung Diseases

Abstract

Nonidiopathic pulmonary fibrosis (non-IPF) progressive fibrotic interstitial lung diseases (PF-ILDs) are a heterogeneous group of ILDs, often challenging to diagnose, although an accurate diagnosis has significant implications for both treatment and prognosis. A subgroup of these patients experiences progressive deterioration in lung function, physical performance, and quality of life after conventional therapy. Risk factors for ILD progression include older age, lower baseline pulmonary function, and a usual interstitial pneumonia pattern. Management of non-IPF P-ILD is both pharmacologic and nonpharmacologic. Antifibrotic drugs, originally approved for IPF, have been considered in patients with other fibrotic ILD subtypes, with favorable results in clinical trials.

Keywords: Antifibrotic treatment; Connective tissue disease-interstitial lung disease; Fibrotic hypersensitivity pneumonitis; Idiopathic pulmonary fibrosis; Nintedanib; Pirfenidone; Progressive fibrotic interstitial lung disease; Unclassifiable interstitial lung disease.