Interstitial lung diseases (ILDs) encompass a large and varied group of parenchymal lung disorders, including diseases of unknown cause, which are known as idiopathic interstitial pneumonias, as well as those associated with other diseases or environmental exposures. The most extensively studied subtype of ILD is idiopathic pulmonary fibrosis (IPF).
A proportion of patients with other non-IPF subtypes of ILD can develop a progressive fibrosing phenotype that shows similarities in underlying mechanisms and clinical behavior to IPF. These progressive fibrosing ILDs (PF-ILDs), while diverse in etiology, share a similar clinical phenotype, accelerated respiratory failure, frequent disease exacerbation, and earlier mortality, and progress through similar mechanisms of self-sustained dysregulated cell repair, fibroblast proliferation, and alveolar dysfunction that can be targeted therapeutically.
ILDs associated with a progressive fibrosing phenotype include connective tissue disease-related ILDs (CTD-ILDs), such as those related to rheumatoid arthritis (RA-ILD), systemic sclerosis, and polymyositis/dermatomyositis, and ILDs related to chronic sarcoidosis, chronic hypersensitivity pneumonitis (HP), idiopathic nonspecific interstitial pneumonia, and unclassifiable ILD.
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Cite this: Zab Mosenifar. Fast Five Quiz: Progressive Fibrosing Interstitial Lung Disease - Medscape - Apr 12, 2022.