PT - JOURNAL ARTICLE AU - Cottin, Vincent AU - Hirani, Nikhil A. AU - Hotchkin, David L. AU - Nambiar, Anoop M. AU - Ogura, Takashi AU - Otaola, MarĂ­a AU - Skowasch, Dirk AU - Park, Jong Sun AU - Poonyagariyagorn, Hataya K. AU - Wuyts, Wim AU - Wells, Athol U. TI - Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases AID - 10.1183/16000617.0076-2018 DP - 2018 Dec 31 TA - European Respiratory Review PG - 180076 VI - 27 IP - 150 4099 - https://publications.ersnet.org//content/27/150/180076.short 4100 - https://publications.ersnet.org//content/27/150/180076.full SO - EUROPEAN RESPIRATORY REVIEW2018 Dec 31; 27 AB - Although these conditions are rare, a proportion of patients with interstitial lung diseases (ILDs) may develop a progressive-fibrosing phenotype. Progressive fibrosis is associated with worsening respiratory symptoms, lung function decline, limited response to immunomodulatory therapies, decreased quality of life and, potentially, early death. Idiopathic pulmonary fibrosis may be regarded as a model for other progressive-fibrosing ILDs. Here we focus on other ILDs that may present a progressive-fibrosing phenotype, namely idiopathic nonspecific interstitial pneumonia, unclassifiable idiopathic interstitial pneumonia, connective tissue disease-associated ILDs (e.g. rheumatoid arthritis-related ILD), fibrotic chronic hypersensitivity pneumonitis, fibrotic chronic sarcoidosis and ILDs related to other occupational exposures. Differential diagnosis of these ILDs can be challenging, and requires detailed consideration of clinical, radiological and histopathological features. Accurate and early diagnosis is crucial to ensure that patients are treated optimally.Other chronic ILDs with a progressive-fibrosing phenotype may have a clinical course similar to IPF. Although challenging, identification of these patients is crucial, and requires a multidisciplinary approach, to ensure optimal diagnosis and management. http://ow.ly/8q8M30mGDsQ