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

Research output: Contribution to journalArticlepeer-review

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 languageEnglish
JournalEuropean Respiratory Journal
Early online date3 Jan 2019
Publication statusE-pub ahead of print - 3 Jan 2019


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