Open AccessIdiopathic and immune‐related pulmonary fibrosis: diagnostic and therapeutic challenges
Author(s) -
McLeanTooke Andrew,
Moore Irene,
Lake Fiona
Publication year - 2019
Publication title -
clinical and translational immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.321
H-Index - 34
ISSN - 2050-0068
DOI - 10.1002/cti2.1086
Subject(s) - medicine , genetic predisposition , interstitial lung disease , idiopathic pulmonary fibrosis , pulmonary fibrosis , idiopathic interstitial pneumonia , hypersensitivity pneumonitis , disease , lung , fibrosis , intensive care medicine , pathology
Interstitial lung disease (ILD) encompasses a large group of pulmonary conditions sharing common clinical, radiological and histopathological features as a consequence of fibrosis of the lung interstitium. The majority of ILDs are idiopathic in nature with possible genetic predisposition, but is also well recognised as a complication of connective tissue disease or with certain environmental, occupational or drug exposures. In recent years, a concerted international effort has been made to standardise the diagnostic criteria in ILD subtypes, formalise multidisciplinary pathways and standardise treatment recommendations. In this review, we discuss some of the current challenges around ILD diagnostics, the role of serological testing, especially, in light of the new classification of Interstitial Pneumonia with Autoimmune Features (IPAF) and discuss the evidence for therapies targeted at idiopathic and immune‐related pulmonary fibrosis.