Predicting outcomes in rheumatoid arthritis related interstitial lung disease

Joseph Jacob; Nikhil Hirani; Coline H M van Moorsel; Srinivasan Rajagopalan; John T Murchison; Hendrik W van Es; Brian J Bartholmai; Frouke T van Beek; Marjolijn H L Struik; Gareth A Stewart; Maria Kokosi; Ryoko Egashira; Anne Laure Brun; Gary Cross; Joseph Barnett; Anand Devaraj; George Margaritopoulos; Ronald Karwoski; Elisabetta Renzoni; Toby M Maher; Athol U Wells

doi:10.1183/13993003.00869-2018

Predicting outcomes in rheumatoid arthritis related interstitial lung disease

Joseph Jacob, Nikhil Hirani, Coline H M van Moorsel, Srinivasan Rajagopalan, John T Murchison, Hendrik W van Es, Brian J Bartholmai, Frouke T van Beek, Marjolijn H L Struik, Gareth A Stewart, Maria Kokosi, Ryoko Egashira, Anne Laure Brun, Gary Cross, Joseph Barnett, Anand Devaraj, George Margaritopoulos, Ronald Karwoski, Elisabetta Renzoni, Toby M MaherAthol U Wells

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Abstract / Description of output

Aims: To compare radiology-based prediction models in rheumatoid arthritis-related interstitial lung disease (RA-ILD) to identify patients with a progressive fibrosis phenotype.Methods: RAILD patients had CTs scored visually and by CALIPER and forced vital capacity (FVC) measurements. Outcomes were evaluated using three techniques: 1.Scleroderma system evaluating visual ILD extent and FVC values; 2.Fleischer Society IPF diagnostic guidelines applied to RAILD; 3.CALIPER scores of vessel-related structures (VRS). Outcomes were compared to IPF patients.Results: On univariable Cox analysis, all three staging systems strongly predicted outcome: Scleroderma System:HR=3.78, p=9×10-5; Fleischner System:HR=1.98, p=2×10-3; 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, gender and smoking status, when analysed alongside the Progressive Fibrotic System, the VRS threshold of 4.4% independently predicted outcome (Model C-statistic=0.77).Conclusions: 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.

Original language	English
Journal	European Respiratory Journal
Early online date	3 Jan 2019
DOIs	https://doi.org/10.1183/13993003.00869-2018
Publication status	E-pub ahead of print - 3 Jan 2019

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Jacob, J., Hirani, N., van Moorsel, C. H. M., Rajagopalan, S., Murchison, J. T., van Es, H. W., Bartholmai, B. J., van Beek, F. T., Struik, M. H. L., Stewart, G. A., Kokosi, M., Egashira, R., Brun, A. L., Cross, G., Barnett, J., Devaraj, A., Margaritopoulos, G., Karwoski, R., Renzoni, E., ... Wells, A. U. (2019). Predicting outcomes in rheumatoid arthritis related interstitial lung disease. European Respiratory Journal. Advance online publication. https://doi.org/10.1183/13993003.00869-2018

@article{c8703874a84744f9b88cc3474affb2b3,

title = "Predicting outcomes in rheumatoid arthritis related interstitial lung disease",

abstract = "Aims: To compare radiology-based prediction models in rheumatoid arthritis-related interstitial lung disease (RA-ILD) to identify patients with a progressive fibrosis phenotype.Methods: RAILD patients had CTs scored visually and by CALIPER and forced vital capacity (FVC) measurements. Outcomes were evaluated using three techniques: 1.Scleroderma system evaluating visual ILD extent and FVC values; 2.Fleischer Society IPF diagnostic guidelines applied to RAILD; 3.CALIPER scores of vessel-related structures (VRS). Outcomes were compared to IPF patients.Results: On univariable Cox analysis, all three staging systems strongly predicted outcome: Scleroderma System:HR=3.78, p=9×10-5; Fleischner System:HR=1.98, p=2×10-3; 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, gender and smoking status, when analysed alongside the Progressive Fibrotic System, the VRS threshold of 4.4% independently predicted outcome (Model C-statistic=0.77).Conclusions: 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.",

author = "Joseph Jacob and Nikhil Hirani and {van Moorsel}, {Coline H M} and Srinivasan Rajagopalan and Murchison, {John T} and {van Es}, {Hendrik W} and Bartholmai, {Brian J} and {van Beek}, {Frouke T} and Struik, {Marjolijn H L} and Stewart, {Gareth A} and Maria Kokosi and Ryoko Egashira and Brun, {Anne Laure} and Gary Cross and Joseph Barnett and Anand Devaraj and George Margaritopoulos and Ronald Karwoski and Elisabetta Renzoni and Maher, {Toby M} and Wells, {Athol U}",

note = "Copyright {\textcopyright}ERS 2018.",

year = "2019",

month = jan,

day = "3",

doi = "10.1183/13993003.00869-2018",

language = "English",

journal = "European Respiratory Journal",

issn = "0903-1936",

publisher = "European Respiratory Society",

}

Jacob, J, Hirani, N, van Moorsel, CHM, Rajagopalan, S, Murchison, JT, van Es, HW, Bartholmai, BJ, van Beek, FT, Struik, MHL, Stewart, GA, Kokosi, M, Egashira, R, Brun, AL, Cross, G, Barnett, J, Devaraj, A, Margaritopoulos, G, Karwoski, R, Renzoni, E, Maher, TM & Wells, AU 2019, 'Predicting outcomes in rheumatoid arthritis related interstitial lung disease', European Respiratory Journal. https://doi.org/10.1183/13993003.00869-2018

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

PY - 2019/1/3

Y1 - 2019/1/3

N2 - Aims: To compare radiology-based prediction models in rheumatoid arthritis-related interstitial lung disease (RA-ILD) to identify patients with a progressive fibrosis phenotype.Methods: RAILD patients had CTs scored visually and by CALIPER and forced vital capacity (FVC) measurements. Outcomes were evaluated using three techniques: 1.Scleroderma system evaluating visual ILD extent and FVC values; 2.Fleischer Society IPF diagnostic guidelines applied to RAILD; 3.CALIPER scores of vessel-related structures (VRS). Outcomes were compared to IPF patients.Results: On univariable Cox analysis, all three staging systems strongly predicted outcome: Scleroderma System:HR=3.78, p=9×10-5; Fleischner System:HR=1.98, p=2×10-3; 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, gender and smoking status, when analysed alongside the Progressive Fibrotic System, the VRS threshold of 4.4% independently predicted outcome (Model C-statistic=0.77).Conclusions: 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 - Aims: To compare radiology-based prediction models in rheumatoid arthritis-related interstitial lung disease (RA-ILD) to identify patients with a progressive fibrosis phenotype.Methods: RAILD patients had CTs scored visually and by CALIPER and forced vital capacity (FVC) measurements. Outcomes were evaluated using three techniques: 1.Scleroderma system evaluating visual ILD extent and FVC values; 2.Fleischer Society IPF diagnostic guidelines applied to RAILD; 3.CALIPER scores of vessel-related structures (VRS). Outcomes were compared to IPF patients.Results: On univariable Cox analysis, all three staging systems strongly predicted outcome: Scleroderma System:HR=3.78, p=9×10-5; Fleischner System:HR=1.98, p=2×10-3; 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, gender and smoking status, when analysed alongside the Progressive Fibrotic System, the VRS threshold of 4.4% independently predicted outcome (Model C-statistic=0.77).Conclusions: 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.

U2 - 10.1183/13993003.00869-2018

DO - 10.1183/13993003.00869-2018

M3 - Article

C2 - 30487199

SN - 0903-1936

JO - European Respiratory Journal

JF - European Respiratory Journal

ER -

Predicting outcomes in rheumatoid arthritis related interstitial lung disease

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