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Predictors of progression in systemic sclerosis patients with interstitial lung disease

Distler, O; Assassi, S; Cottin, V; Cutolo, M; Danoff, SK; Denton, CP; Distler, JHW; ... Maher, TM; + view all (2020) Predictors of progression in systemic sclerosis patients with interstitial lung disease. European Respiratory Journal , 55 (5) , Article 1902026. 10.1183/13993003.02026-2019. Green open access

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Abstract

Systemic sclerosis (SSc) is a systemic autoimmune disease affecting multiple organ systems, including the lungs. Interstitial lung disease (ILD) is the leading cause of death in SSc. There are no valid biomarkers to predict the occurrence of SSc-ILD, although auto-antibodies against anti-topoisomerase I and several inflammatory markers are candidate biomarkers that need further evaluation. Chest auscultation, presence of shortness of breath and pulmonary function testing are important diagnostic tools, but lack sensitivity to detect early ILD. Baseline screening with high-resolution computed tomography (HRCT) is therefore necessary to confirm an SSc-ILD diagnosis. Once diagnosed with SSc-ILD, patients' clinical courses are variable and difficult to predict, though certain patient characteristics and biomarkers are associated with disease progression. It is important to monitor patients with SSc-ILD for signs of disease progression, though there is no consensus about which diagnostic tools to use or how often monitoring should occur. In this article, we review methods used to define and predict disease progression in SSc-ILD. There is no valid definition of SSc-ILD disease progression, but we suggest that either a decline in forced vital capacity (FVC) from baseline of ≥10%, or an FVC decline of 5–9% in association with a decline in diffusing capacity of carbon monoxide of ≥15% represents progression. An increase in the radiographic extent of ILD on HRCT imaging would also signify progression. A time period of 1–2 years is generally used for this definition, but a decline over a longer time period may also reflect clinically relevant disease progression.

Type: Article
Title: Predictors of progression in systemic sclerosis patients with interstitial lung disease
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1183/13993003.02026-2019
Publisher version: https://doi.org/10.1183/13993003.02026-2019
Language: English
Additional information: Copyright © ERS 2020. This version is distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: https://discovery.ucl.ac.uk/id/eprint/10092075
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