Interstitial lung diseases (ILD) are a heterogenous group of respiratory disorders with varying degrees of inflammation and fibrosis. Idiopathic pulmonary fibrosis (IPF), the commonest and most debilitating type of ILD, is a chronic, progressive disease of the respiratory system characterized by fibrosis of the alveolar interstitium. Subsequent, relentless decline in lung function leads to progressive breathlessness and respiratory failure. Treatment options for IPF have remained mostly unchanged in the last decade, with the availability of two antifibrotic therapies: pirfenidone and nintedanib. Recently, the US Food and Drugs Administration (FDA) approved nerandomilast for the management of IPF and progressive pulmonary fibrosis. Nintedanib is also globally approved for the treatment of progressive non-IPF ILDs. While these therapies have been shown to reduce the decline of lung function, they do not reverse existing lung damage or fully address the complex pathophysiology of pulmonary fibrosis (PF). Accordingly, research in the field has shifted to developing new therapies with improved efficacy and minimal adverse effects that directly target the intricate pathogenesis in PF with the aim of arresting or reversing the disease. This review article will set the scene by first describing the pathogenesis and prevalence of ILDs, followed by exploring the current and emerging therapies in the field.
Etchingham-Coll et al. (Tue,) studied this question.