TY - JOUR
T1 - Predicting outcomes in rheumatoid arthritis related interstitial lung disease
AU - Jacob, Joseph
AU - Hirani, Nikhil
AU - Van Moorsel, Coline H.M.
AU - Rajagopalan, Srinivasan
AU - Murchison, John T.
AU - Van Es, Hendrik W.
AU - Bartholmai, Brian J.
AU - Van Beek, Frouke T.
AU - Struik, Marjolijn H.L.
AU - Stewart, Gareth A.
AU - Kokosi, Maria
AU - Egashira, Ryoko
AU - Brun, Anne Laure
AU - Cross, Gary
AU - Barnett, Joseph
AU - Devaraj, Anand
AU - Margaritopoulos, George
AU - Karwoski, Ronald
AU - Renzoni, Elisabetta
AU - Maher, Toby M.
AU - Wells, Athol U.
N1 - Funding Information:
Support statement: The work was supported by the National Institute of Health Research Respiratory Disease Biomedical Research Unit at the Royal Brompton and Harefield NHS Foundation Trust and Imperial College London. J. Jacob was supported by a Wellcome Trust Clinical Research Career Development Fellowship 209553/Z/17/Z.
Publisher Copyright:
Copyright ©ERS 2019.
PY - 2019/1/1
Y1 - 2019/1/1
N2 - The aim of this study was to compare radiology-based prediction models in rheumatoid arthritis-related interstitial lung disease (RAILD) to identify patients with a progressive fibrosis phenotype. RAILD patients had computed tomography (CT) scans scored visually and using CALIPER and forced vital capacity (FVC) measurements. Outcomes were evaluated using three techniques, as follows. 1) Scleroderma system evaluating visual interstitial lung disease extent and FVC values; 2) Fleischner Society idiopathic pulmonary fibrosis (IPF) diagnostic guidelines applied to RAILD; and 3) CALIPER scores of vessel-related structures (VRS). Outcomes were compared to IPF patients. On univariable Cox analysis, all three staging systems strongly predicted outcome (scleroderma system hazard ratio (HR) 3.78, p=9×10 −5 ; Fleischner system HR 1.98, p=2×10 −3 ; and 4.4% VRS threshold HR 3.10, p=4×10 −4 ). When the scleroderma and Fleischner systems were combined, termed the progressive fibrotic system (C-statistic 0.71), they identified a patient subset (n=36) with a progressive fibrotic phenotype and similar 4-year survival to IPF. On multivariable analysis, with adjustment for patient age, sex and smoking status, when analysed alongside the progressive fibrotic system, the VRS threshold of 4.4% independently predicted outcome (model C-statistic 0.77). The combination of two visual CT-based staging systems identified 23% of an RAILD cohort with an IPF-like progressive fibrotic phenotype. The addition of a computer-derived VRS threshold further improved outcome prediction and model fit, beyond that encompassed by RAILD measures of disease severity and extent.
AB - The aim of this study was to compare radiology-based prediction models in rheumatoid arthritis-related interstitial lung disease (RAILD) to identify patients with a progressive fibrosis phenotype. RAILD patients had computed tomography (CT) scans scored visually and using CALIPER and forced vital capacity (FVC) measurements. Outcomes were evaluated using three techniques, as follows. 1) Scleroderma system evaluating visual interstitial lung disease extent and FVC values; 2) Fleischner Society idiopathic pulmonary fibrosis (IPF) diagnostic guidelines applied to RAILD; and 3) CALIPER scores of vessel-related structures (VRS). Outcomes were compared to IPF patients. On univariable Cox analysis, all three staging systems strongly predicted outcome (scleroderma system hazard ratio (HR) 3.78, p=9×10 −5 ; Fleischner system HR 1.98, p=2×10 −3 ; and 4.4% VRS threshold HR 3.10, p=4×10 −4 ). When the scleroderma and Fleischner systems were combined, termed the progressive fibrotic system (C-statistic 0.71), they identified a patient subset (n=36) with a progressive fibrotic phenotype and similar 4-year survival to IPF. On multivariable analysis, with adjustment for patient age, sex and smoking status, when analysed alongside the progressive fibrotic system, the VRS threshold of 4.4% independently predicted outcome (model C-statistic 0.77). The combination of two visual CT-based staging systems identified 23% of an RAILD cohort with an IPF-like progressive fibrotic phenotype. The addition of a computer-derived VRS threshold further improved outcome prediction and model fit, beyond that encompassed by RAILD measures of disease severity and extent.
UR - http://www.scopus.com/inward/record.url?scp=85059275754&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85059275754&partnerID=8YFLogxK
U2 - 10.1183/13993003.00869-2018
DO - 10.1183/13993003.00869-2018
M3 - Article
C2 - 30487199
AN - SCOPUS:85059275754
SN - 0903-1936
VL - 53
JO - European Respiratory Journal
JF - European Respiratory Journal
IS - 1
M1 - 1800869
ER -