Imperial College London
Alternate

Professor Toby Maher

Faculty of Medicine, National Heart & Lung Institute

Professor of Interstitial Lung Disease
 
 
 
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Contact

 

+44 (0)20 7594 2151t.maher

 
 
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Assistant

 

Ms Georgina Moss +44 (0)20 7594 2151

 
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Location

 

364Sir Alexander Fleming BuildingSouth Kensington Campus

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Summary

 

Publications

Citation

BibTex format

@article{Distler:2020:10.1183/13993003.02026-2019,
author = {Distler, O and Assassi, S and Cottin, V and Cutolo, M and Danoff, SK and Denton, CP and Distler, JHW and Hoffmann-Vold, A-M and Johnson, SR and Müller, Ladner U and Smith, V and Volkmann, ER and Maher, TM},
doi = {10.1183/13993003.02026-2019},
journal = {European Respiratory Journal},
title = {Predictors of progression in systemic sclerosis patients with interstitial lung disease},
url = {http://dx.doi.org/10.1183/13993003.02026-2019},
volume = {55},
year = {2020}
}
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RIS format (EndNote, RefMan)

TY  - JOUR
AB  - 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 1-2years is generally used for this definition, but a decline over a longer time period may also reflect clinically relevant disease progression.
AU  - Distler,O
AU  - Assassi,S
AU  - Cottin,V
AU  - Cutolo,M
AU  - Danoff,SK
AU  - Denton,CP
AU  - Distler,JHW
AU  - Hoffmann-Vold,A-M
AU  - Johnson,SR
AU  - Müller,Ladner U
AU  - Smith,V
AU  - Volkmann,ER
AU  - Maher,TM
DO  - 10.1183/13993003.02026-2019
PY  - 2020///
SN  - 0903-1936
TI  - Predictors of progression in systemic sclerosis patients with interstitial lung disease
T2  - European Respiratory Journal
UR  - http://dx.doi.org/10.1183/13993003.02026-2019
UR  - https://www.ncbi.nlm.nih.gov/pubmed/32079645
UR  - http://hdl.handle.net/10044/1/77773
VL  - 55
ER  -
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