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.
Funding Information:
Conflict of interest: J. Jacob reports advisory board fees from Boehringer Ingelheim, outside the submitted work. N. Hirani reports personal fees from Boehringer Ingelheim, Intermune, Roche, Galecto and UCB, outside the submitted work. C.H.M. van Moorsel has nothing to disclose. S. Rajagopalan reports grants (provided to Mayo Clinic for supporting the handling and processing of the CT datasets used in the study) from Royal Brompton Hospital, during the conduct of the study; and that Mayo Clinic has received royalties from Imbio, LCC towards licensing CALIPER, outside the submitted work; in addition, S. Rajagopalan has a patent Systems And Methods For Analysing In Vivo Tissue Volumes Using Medical Imaging Data licensed to Imbio, LLC. J.T. Murchison has nothing to disclose. H.W. van Es has nothing to disclose. B.J. Bartholmai reports grants (provided to Mayo Clinic for supporting the handling and processing of CT datasets used in the study) from Royal Brompton Hospital, during the conduct of the study; and that Mayo Clinic has received royalties from Imbio, LCC towards licensing CALIPER, outside the submitted work; in addition, B.J. Bartholmai has a patent Systems And Methods For Analysing In Vivo Tissue Volumes Using Medical Imaging Data licensed to Imbio, LLC. F.T. van Beek has nothing to disclose. M.H.L. Struik has nothing to disclose. G.A. Stewart has nothing to disclose. M. Kokosi has nothing to disclose. R. Egashira has nothing to disclose. A.L. Brun has nothing to disclose. G. Cross has nothing to disclose. J. Barnett has nothing to disclose. A. Devaraj reports personal fees from Roche and Boehringer Ingelheim, outside the submitted work. G. Margaritopoulos has nothing to disclose. R. Karwoski reports grants (provided to Mayo Clinic for supporting the handling and processing of CT datasets used in the study) from Royal Brompton Hospital, during the conduct of the study; and that Mayo Clinic has received royalties from Imbio, LCC towards licensing CALIPER, outside the submitted work; in addition, R. Karwoski has a patent Systems And Methods For Analysing In Vivo Tissue Volumes Using Medical Imaging Data licensed to Imbio, LLC. E. Renzoni reports lecture fees from Roche and Takeda, and lecture fees and advisory board fees from Boehringer, outside the submitted work. T.M. Maher has, via his institution, received industry-academic funding from GlaxoSmithKline R&D, UCB and Novartis and has received consultancy or speakers fees from Apellis, Astra Zeneca, Bayer, Biogen Idec, Boehringer Ingelheim, Cipla, GlaxoSmithKline R&D, Lanthio, InterMune, ProMetic, Roche, Sanofi-Aventis, Takeda and UCB. A.U. Wells reports advisory board and speaker fees from Intermune, Boehringer Ingelheim, Roche and Bayer, advisory board fees from Gilead and MSD, and speaker fees from Chiesi, outside the submitted work.
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.
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U2 - 10.1183/13993003.00869-2018
DO - 10.1183/13993003.00869-2018
M3 - Article
C2 - 30487199
AN - SCOPUS:85059275754
VL - 53
JO - Scandinavian Journal of Respiratory Diseases
JF - Scandinavian Journal of Respiratory Diseases
SN - 0903-1936
IS - 1
M1 - 1800869
ER -