Abstract
A significant proportion of patients with interstitial lung disease (ILD) may develop a progressive fibrosing phenotype characterized by worsening of symptoms and pulmonary function, progressive fibrosis on chest computed tomography and increased mortality. The clinical course in these patients mimics the relentless progressiveness of idiopathic pulmonary fibrosis (IPF). Common pathophysiological mechanisms such as a shared genetic susceptibility and a common downstream pathway—self-sustaining fibroproliferation—support the concept of a progressive fibrosing phenotype, which is applicable to a broad range of non-IPF ILDs. While antifibrotic drugs became the standard of care in IPF, immunosuppressive agents are still the mainstay of treatment in non-IPF fibrosing ILD (F-ILD). However, recently, randomized placebo-controlled trials have demonstrated the efficacy and safety of antifibrotic treatment in systemic sclerosis-associated F-ILD and a broad range of F-ILDs with a progressive phenotype. This review summarizes the current pharmacological management and highlights the unmet needs in patients with non-IPF ILD.
Highlights
In addition to idiopathic pulmonary fibrosis (IPF), which is defined as a relentless progressive disease, a significant proportion of patients with other interstitial lung disease (ILD) subtypes may develop a progressive phenotype characterized by worsening of symptoms, deterioration of pulmonary function, progressive pulmonary fibrosis on chest computed tomography (CT) and increased mortality
Other non-IPF ILD subtypes that may be associated with a progressive fibrosing phenotype include connective tissue disease-associated ILD (CTD-ILD), idiopathic non-specific interstitial pneumonia, sarcoidosis, hypersensitivity pneumonitis (HP), occupational ILD and unclassifiable ILD
Corticosteroids and other immunosuppressive agents such as AZA, CYC and mycophenolate mofetil (MMF) have always been the cornerstone of management in interstitial pneumonia (iNSIP), but no randomized controlled trials (RCTs) are available, and only a few retrospective cohort studies have investigated the efficacy of immunosuppressive treatment in iNSIP patients
Summary
Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. In addition to idiopathic pulmonary fibrosis (IPF), which is defined as a relentless progressive disease, a significant proportion of patients with other interstitial lung disease (ILD) subtypes may develop a progressive phenotype characterized by worsening of symptoms, deterioration of pulmonary function, progressive pulmonary fibrosis on chest computed tomography (CT) and increased mortality. Other non-IPF ILD subtypes that may be associated with a progressive fibrosing phenotype include connective tissue disease-associated ILD (CTD-ILD), idiopathic non-specific interstitial pneumonia (iNSIP), sarcoidosis, hypersensitivity pneumonitis (HP), occupational ILD and unclassifiable ILD (uILD) [1,2,3,4]. Based on survey data and cohort studies, it is estimated that 30% of non-IPF. We summarize the current pharmacological management and highlight the unmet needs in patients with non-IPF ILD
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