Advances with pharmacotherapy for the treatment of interstitial lung disease

Alessia Comes, Luca Richeldi, Giacomo Sgalla, Alessandro Perrotta

Research output: Contribution to journalArticle


Introduction: In recent decades, the primary focus of pharmaceutical research in interstitial lung diseases (ILD) has been on idiopathic pulmonary fibrosis (IPF). Recently, pharmaceutical development has also focused on other forms of ILDs, including connective tissue diseases associated ILD, fibrotic hypersensitivity pneumonitis, and sarcoidosis. Areas Covered: The authors summarize the advances in pharmacotherapy for the treatment of ILD. Specifically, the authors review the most recent studies and discuss the most recent research findings and future prospects. Expert opinion: Data collected over the past years have confirmed the efficacy of antifibrotic drugs on slowing disease progression in IPF. The usual strategy for CTD-ILD management is represented by the combined use of corticosteroids and immunosuppressive agents. There is an urgent need for new target therapies. The concept of progressive fibrosing ILD has emerged in the ILD community in recent years, which has led to grouping several diseases with a common disease behavior to find an effective treatment. At present, selecting the best therapy in ILDs should be reasonably performed on a case-by-case basis through a multidisciplinary team discussion in tertiary ILD centers, taking into consideration patients’ symptoms, lung functional trends, and radiological changes.
Original languageEnglish
Pages (from-to)483-495
Number of pages13
Publication statusPublished - 2022


  • connective tissue disease associated ILD
  • Sarcoidosis
  • idiopathic pulmonary fibrosis
  • Interstitial lung disease
  • nintedanib
  • pirfenidone
  • progressive fibrosing ILD
  • sarcoidosis
  • Disease Progression
  • Fibrosis
  • Humans
  • Lung
  • Idiopathic Pulmonary Fibrosis
  • Lung Diseases, Interstitial
  • fibrotic hypersensitivity pneumonitis


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