Author(s): Cotton C.V.; New R.P.; Cooper R.G.; Spencer L.G.
Source: Rheumatology (United Kingdom); Aug 2017; vol. 56 (no. 8); p. 1264-1271
Publication Date: Aug 2017
Publication Type(s): Review
Abstract:Interstitial lung disease (ILD) comprises many heterogeneous disease groups, the largest being CTD-associated and those labelled as idiopathic out of necessity. The mechanisms causing ILD are poorly understood, but most CTD- and idiopathic-ILD cases can respond to immunosuppression, clearly suggesting a pathological role for inflammation. By contrast, corticosteroid immunosuppression causes harm without benefit in the feared idiopathic pulmonary fibrosis, suggesting that inflammation plays little pathological role, and where ILD progresses rapidly to lethal outcome even with anti-fibrotic drug use. Given the treatment response differences apparent between ILD subgroups, and the dangers and costs of corticosteroid and anti-fibrotic drug use, respectively, it has become vital in every ILD patient to make an accurate subgroup diagnosis, to optimize treatment selections. This review discusses why differentiating CTD- and idiopathic-ILD subgroup cases remains so problematic, and why existing comprehensive CTD-specific serology would, if generally available, represent an ideal biomarker tool to enhance ILD subgroup diagnostic accuracy. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved.
Copyright © The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved.