PT  - JOURNAL ARTICLE
AU  - Vincent Cottin
AU  - Nikhil A. Hirani
AU  - David L. Hotchkin
AU  - Anoop M. Nambiar
AU  - Takashi Ogura
AU  - María Otaola
AU  - Dirk Skowasch
AU  - Jong Sun Park
AU  - Hataya K. Poonyagariyagorn
AU  - Wim Wuyts
AU  - Athol U. Wells
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  - http://err.ersjournals.com/content/27/150/180076.short
4100  - http://err.ersjournals.com/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