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{Stock:2021:10.1111/resp.13988,
author = {Stock, CJW and Hoyles, RK and Daccord, C and Kokosi, M and Visca, D and De, Lauretis A and Alfieri, V and Kouranos, V and Margaritopoulos, G and George, PM and Molyneaux, PL and Chua, F and Maher, TM and Abraham, DJ and Ong, V and Donovan, J and Sestini, P and Denton, CP and Wells, AU and Renzoni, EA},
doi = {10.1111/resp.13988},
journal = {Respirology},
pages = {461--468},
title = {Serum markers of pulmonary epithelial damage in systemic sclerosis-associated interstitial lung disease and disease progression},
url = {http://dx.doi.org/10.1111/resp.13988},
volume = {26},
year = {2021}
}
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RIS format (EndNote, RefMan)

TY  - JOUR
AB  - Background and objectiveThe course of systemic sclerosisassociated interstitial lung disease (SScILD) is highly variable, and accurate prognostic markers are needed. KL6 is a mucinlike glycoprotein (MUC1) expressed by type II pneumocytes, while CYFRA 211 is expressed by alveolar and bronchiolar epithelial cells. Both are released into the blood from cell injury.MethodsSerum KL6 and CYFRA 211 levels were measured in a retrospective (n = 189) and a prospective (n = 118) cohort of SSc patients. Genotyping of MUC1 rs4072037 was performed. Linear mixedeffect models were used to evaluate the relationship with change in lung function parameters over time, while association with survival was evaluated with Cox proportional hazard analysis.ResultsIn both cohorts, KL6 and CYFRA 211 were highest in patients with lung involvement, and in patients with extensive rather than limited ILD. KL6 was higher in patients carrying the MUC1 rs4072037 G allele in both cohorts. In patients with SScILD, serum KL6, but not CYFRA 211, was significantly associated with DLCO decline in both cohorts (P = 0.001 and P = 0.004, respectively), and with FVC decline in the retrospective cohort (P = 0.005), but not the prospective cohort. When combining the cohorts and subgrouping by severity (median CPI = 45.97), KL6 remained predictive of decline in DLCO in both milder (P = 0.007) and more severe disease (P = 0.02) on multivariable analysis correcting for age, gender, ethnicity, smoking history and MUC1 allele carriage.ConclusionOur results suggest serum KL6 predicts decline in lung function in SSc, suggesting its clinical utility in risk stratification for progressive SScILD.
AU  - Stock,CJW
AU  - Hoyles,RK
AU  - Daccord,C
AU  - Kokosi,M
AU  - Visca,D
AU  - De,Lauretis A
AU  - Alfieri,V
AU  - Kouranos,V
AU  - Margaritopoulos,G
AU  - George,PM
AU  - Molyneaux,PL
AU  - Chua,F
AU  - Maher,TM
AU  - Abraham,DJ
AU  - Ong,V
AU  - Donovan,J
AU  - Sestini,P
AU  - Denton,CP
AU  - Wells,AU
AU  - Renzoni,EA
DO  - 10.1111/resp.13988
EP  - 468
PY  - 2021///
SN  - 1323-7799
SP  - 461
TI  - Serum markers of pulmonary epithelial damage in systemic sclerosis-associated interstitial lung disease and disease progression
T2  - Respirology
UR  - http://dx.doi.org/10.1111/resp.13988
UR  - http://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000599523100001&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=1ba7043ffcc86c417c072aa74d649202
UR  - https://onlinelibrary.wiley.com/doi/10.1111/resp.13988
UR  - http://hdl.handle.net/10044/1/86143
VL  - 26
ER  -
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