Περίληψη:
Background: The major risk factor for idiopathic pulmonary fibrosis
(IPF), MUC5B rs35705950, was found to be associated with rheumatoid
arthritis-associated interstitial lung disease (RA-ILD). Whilst the
MUC5B rs35705950 T risk allele has been associated with better survival
in IPF, its impact on RA-ILD prognosis remains to be determined. Our
objective was to explore the influence of MUC5B rs35705950 on survival
and progression in RA-ILD. Methods: Through an international
retrospective observational study, patients with RA-ILD were genotyped
for the MUC5B rs35705950 variant and consecutive pulmonary function
tests (PFTs) findings were collected. Longitudinal data up to a 10-year
follow-up were considered and analyzed using mixed regression models.
Proportional hazards and joint proportional hazards models were used to
analyze the association of baseline and longitudinal variables with lung
transplant-free survival. Significant progression of RA-ILD was defined
as at least an absolute or relative 10% decline of forced vital
capacity at 2 years from baseline. Results: Out of 321 registered
patients, 261 were included in the study: 139 women (53.3%), median age
at RA-ILD diagnosis 65 years (interquartile range [IQR] 57 to 71), 151
ever smokers (59.2%). Median follow-up was 3.5 years (IQR 1.3 to 6.6).
Mortality rate was 32% (95%CI 19 to 42) at 10 years. The MUC5B
rs35705950 variant did not impact lung transplant-free survival (HR for
the T risk allele carriers=1.26; 95%CI 0.61 to 2.62; P=0.53). Decline
in pulmonary function at 2 years was not influenced by MUC5B rs35705950
(OR=0.95; 95%CI 0.44 to 2.05; P=0.89), irrespective of the HRCT
pattern. Conclusion: In this study, the MUC5B rs35705950 promoter
variant did not influence transplant-free survival or decline in
pulmonary function in patients with RA-ILD. (c) 2021 Published by
Elsevier Inc.
Συγγραφείς:
Juge, Pierre-Antoine
Solomon, Joshua J.
van Moorsel, Coline H.
M.
Garofoli, Romain
Lee, Joyce S.
Louis-Sydney, Fabienne and
Rojas-Serrano, Jorge
Gonzalez-Perez, I, Montserrat
Mejia, Mayra
and Buendia-Roldan, Ivette
Falfan-Valencia, Ramces and
Ambrocio-Ortiz, Enrique
Manali, Effrosyni
Papiris, Spyros A. and
Karageorgas, Theofanis
Boumpas, Dimitrios
Antoniou, Katarina M.
and Sidiropoulos, Prodromos
Trachalaki, Athina
Van der Vis,
Joanne J.
Jamnitski, Anna
Grutters, Jan C.
Kannengiesser,
Caroline
Borie, Raphael
Kawano-Dourado, Leticia and
Wemeau-Stervinou, Lidwine
Flipo, Rene-Marc
Nunes, Hilario and
Uzunhan, Yurdagul
Valeyre, Dominique
Saidenberg-Kermanac'h,
Nathalie
Boissier, Marie-Christophe
Richez, Christophe and
Schaeverbeke, Thierry
Doyle, Tracy
Wolters, Paul J.
Debray,
Marie-Pierre
Boileau, Catherine
Porcher, Raphael
Schwartz,
David A.
Crestani, Bruno
Dieude, Philippe
