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.
Footnotes
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Conflict of interest: Dr. Jacob reports personal fees from Boehringer Ingelheim, outside the submitted work.
Conflict of interest: Dr. Hirani reports personal fees from Boehringer Ingelheim, personal fees from Intermune, personal fees from Roche, personal fees from Galecto, personal fees from UCB, outside the submitted work.
Conflict of interest: Dr. van Moorsel has nothing to disclose.
Conflict of interest: Dr. RAJAGOPALAN reports grants from , Royal Brompton Hospital, during the conduct of the study; other from Imbio, LLC, outside the submitted work; In addition, Dr. RAJAGOPALAN has a patent SYSTEMS AND METHODS FOR ANALYZING IN VIVO TISSUE VOLUMES USING MEDICAL IMAGING DATA licensed to Imbio, LLC.
Conflict of interest: Dr. Murchison has nothing to disclose.
Conflict of interest: Dr. van Es has nothing to disclose.
Conflict of interest: Dr. Bartholmai reports grants from Royal Brompton Hospital, during the conduct of the study; other from Imbio, LLC, outside the submitted work; In addition, Dr. Bartholmai has a patent SYSTEMS AND METHODS FOR ANALYZING IN VIVO TISSUE VOLUMES USING MEDICAL IMAGING DATA licensed to Imbio, LLC.
Conflict of interest: Dr. van Beek has nothing to disclose.
Conflict of interest: Dr. Struik has nothing to disclose.
Conflict of interest: Dr. Stewart has nothing to disclose.
Conflict of interest: Dr. Kokosi has nothing to disclose.
Conflict of interest: Dr. Egashira has nothing to disclose.
Conflict of interest: Dr. Brun has nothing to disclose.
Conflict of interest: Dr. Cross has nothing to disclose.
Conflict of interest: Dr. Barnett has nothing to disclose.
Conflict of interest: Dr. Devaraj reports personal fees from Roche, personal fees from Boehringer Ingelheim, outside the submitted work.
Conflict of interest: Dr. Margaritopoulos has nothing to disclose.
Conflict of interest: Dr. Karwoski reports grants from Royal Brompton Hospital, during the conduct of the study; other from Imbio, LLC, outside the submitted work; In addition, Dr. Karwoski has a patent SYSTEMS AND METHODS FOR ANALYZING IN VIVO TISSUE VOLUMES USING MEDICAL IMAGING DATA licensed to Imbio LLC.
Conflict of interest: Dr. Renzoni reports personal fees from Roche, personal fees from Boehringher, personal fees from Takeda, outside the submitted work.
Conflict of interest: TMM 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.
Conflict of interest: Dr. Wells reports personal fees from Intermune, personal fees from Boehringer Inlgeheim, personal fees from Gilead, personal fees from MSD, personal fees from Roche, personal fees from Bayer, personal fees from Chiesi, outside the submitted work.
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