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Background/Purpose: Rheumatoid arthritis (RA) associated interstitial lung disease (ILD) and idiopathic pulmonary fibrosis (IPF) sharephenotypic and genotypic similarities. Recently, in a large international genetic association case-control study, the IPF major risk factor, MUC5Brs35705950 promoter variant was found to be associated with RA-ILD. Regarding IPF, MUC5Brs35705950 T risk allele has been associated with better prognosis. However, the impact of MUC5Brs35705950 on RA-ILD prognosis remains to be determined. Our objective was to explore the influence of MUC5Brs35705950 on pulmonary function tests (PFTs) evolution in patients with RA-ILD.
Methods: Patients with RA-ILD were included from France, USA, Mexico, Greece andtheNetherlands in this observationalinternational study. Patients were genotyped for MUC5Brs35705950 and PFTs data were collected. Longitudinal data up to a 10-year follow-up were considered and analyzed using mixed regression models. We defined a significant deterioration of pulmonary function as an absolute decrease of at least 10% in FVC (expressed in percent of the predicted value) or a relative decrease in FVC of at least 10%, at two years from baseline. Individuals who underwent lung transplantation or who died before 2 years from baseline were considered as experiencing a significant decrease of pulmonary function.
Results: Out of 321 registered patients, 261 could be included in the study: 139 women (53.3%), median age at RA-ILD diagnosis 65 y/o (IQR 57 – 71), 151 ever smokers (59.2%), 128 definite or possible usual interstitial pneumonia (UIP) (53.4%), 74 non-specific interstitial pneumonia (NSIP) (30.8%). Baseline median forced vital capacity (FVC) was 78.2% predicted (IQR 64.2 – 100.7) and Baseline diffusing capacity for carbon monoxide (DLCO) was 56.0% predicted (IQR 46.5 – 72.0). MUC5Brs35705950 minor allele frequency was 27.20%. Median follow-up was 3.5 years. Overall, there was a slight trend to decrease of 0.8% per year (CI95% 0.3 – 1.4). Additionally, an important inter-patient variability of FVC was found at baseline as well as during follow-up. DLOC shows a significant decrease of 1.8% per year on average (CI95% 1.2 – 2.3). A significant deterioration of PFTs at 2 years was observed for 33 of 256 evaluable patients. No baseline characteristics was associated with a significant deterioration of PFTs at 2 years, including the HRCT UIP pattern (OR=0.7; CI95% 0.3 – 1.6). MUC5Brs35705950 T risk allele was not associated with a better or worse deterioration of PFTs (OR=0.7; CI95% 0.2 – 2.2). No difference was found between MUC5Brs35705950 T risk allele carriers and non-carriers for transplant free survival (Figure 1.).
Conclusion: In this international multiethnicobservational study, PFTs from patients with RA-ILD were relatively stable over time andMUC5Brs35705950 T risk allele was not associated with a better or a worse evolution of PFTs.
Figure 1. Transplant-free survival of all patients (A) and according to MUC5B rs35705950 polymorphism (B).
Disclosure: P. Juge, Bristol-Myers Squibb Company, 5; J. Solomon, Pfizer Inc., 2, Boehringer Ingelheim, 2, Roche-Genentech, 9; R. Garofoli, None; J. Lee, None; F. Louis-Sidney, None; E. Ebstein, None; J. Rojas-Serrano, None; M. Gonzalez-Perez, None; M. Mejia, None; I. Buendia-Roldan, None; R. Falfan-Valencia, None; E. Ambrocio-Ortiz, None; E. Manali, None; S. Papiris, None; T. Karageorgas, None; D. Boumpas, None; K. Antoniou, None; C. van Moorsel, None; J. van Der Vis, None; Y. de Man, None; J. Grutters, None; C. Kannengiesser, None; R. Borie, None; L. Wemeau-Stervinou, None; R. Flipo, MSD France, 1, 2, Sanofi-Aventis, 1; H. Nunes, Boehringer Ingelheim, 1, 2, 3, Roche, 1, 2, 3, Galapagos, 1; Y. Uzunhan, None; D. Valeyre, None; N. Saidenberg Kermanac'h, None; M. Boissier, None; C. Richez, Bristol-Myers Squibb, 9, Amgen SAS, 5; T. Schaeverbeke, None; T. Doyle, None; P. Wolters, Boehringer Ingelheim, 2, Roche/Genentech, 2; M. Debray, None; C. Boileau, None; R. Porcher, None; B. Crestani, None; D. Schwartz, None; P. Dieude, None.
Juge P, Solomon J, Garofoli R, Lee J, Louis-Sidney F, Ebstein E, Rojas-Serrano J, Gonzalez-Perez M, Mejia M, Buendia-Roldan I, Falfan-Valencia R, Ambrocio-Ortiz E, Manali E, Papiris S, Karageorgas T, Boumpas D, Antoniou K, van Moorsel C, van Der Vis J, de Man Y, Grutters J, Kannengiesser C, Borie R, Wemeau-Stervinou L, Flipo R, Nunes H, Uzunhan Y, Valeyre D, Saidenberg Kermanac'h N, Boissier M, Richez C, Schaeverbeke T, Doyle T, Wolters P, Debray M, Boileau C, Porcher R, Crestani B, Schwartz D, Dieude P. MUC5B rs35705950 and Rheumatoid Arthritis Associated Interstitial Lung Disease Progression [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 10). https://acrabstracts.org/abstract/muc5b-rs35705950-and-rheumatoid-arthritis-associated-interstitial-lung-disease-progression/. Accessed September 26, 2021.

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The passage contains four hundred and twenty-three "e"s.
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