RT Journal Article
SR Electronic
T1 Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases
JF European Respiratory Review
JO EUROPEAN RESPIRATORY REVIEW
FD European Respiratory Society
SP 180076
DO 10.1183/16000617.0076-2018
VO 27
IS 150
A1 Vincent Cottin
A1 Nikhil A. Hirani
A1 David L. Hotchkin
A1 Anoop M. Nambiar
A1 Takashi Ogura
A1 María Otaola
A1 Dirk Skowasch
A1 Jong Sun Park
A1 Hataya K. Poonyagariyagorn
A1 Wim Wuyts
A1 Athol U. Wells
YR 2018
UL http://err.ersjournals.com/content/27/150/180076.abstract
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