Sex influence on outcomes of patients with systemic sclerosis-associated interstitial lung disease: A EUSTAR database analysis

Corrado Campochiaro, Anna Maria Hoffmann-Vold, Jerome Avouac, Jörg Henes, Jeska De Vries-Bouwstra, Vanessa Smith, Elise Siegert, Paolo Airò, Fahrettin Oksel, Raffaele Pellerito, Marie Vanthuyne, Maria Rosa Pozzi, Murat Inanc, Jean Sibilia, Armando Gabrielli, Oliver Distler, Yannick Allanore*, Marco Matucci Cerinic, Ulrich Walker, Florenzo IannoneRadim Becvar, Giovanna Cuomo, C. Montecucco, Patricia E. Carreira, Michele Iudici, Eugene J. Kucharz, Elisabetta Zanatta, Pr Dominique Farge Bancel, Roger Hesselstrand, Alexandra Balbir-Gurman, Raffaele Pellerito, Eugenia Bertoldo, Nemanja Damjanov, Vera Ortiz Santamaria Granollers, Stefan Heitmann, Maria João Salvador, Bojana Stamenkovic, Carlo Francesco Selmi, Ariane Herrick, Ulf Mü Ller-Ladner, Merete Engelhart, Valeria Riccieri, Ruxandra Maria Ionescu, Ana Maria Gheorghiu, Cord Sunderkötter, Jörg Distler, Francesca Ingegnoli, Luc Mouthon, Francesco Paolo Cantatore, Susanne Ullman, Piotr Wiland, Marie Vanthuyne, Petra Saar, Kristine Herrmann, Ellen De Langhe, Miroslav Mayer, Sule Yavuz, Carolina De Souza Müller, Thierry Zenone, Alessandra Vacca, Kamal Solanki, Edoardo Rosato, Fahrettin Oksel Figen Yargucu, Cristina Mihaela Tanaseanu, Rosario Foti, Daniel E. Furst, Peter Villiger Sabine Adler, Jorge Juan González Martín, Ira Litinsky, Francesco Del Galdo, Goda Seskute, Lesley Ann Saketkoo, Eduardo Kerzberg, Ivan Castellví, François Spertini, Vivien M. Hsu, Thierry Martin, Tim Schmeiser, Dominik Majewski, Vera Bernardino, Piercarlo Sarzi Puttini, Gianluca Moroncini, Jiri Stork, Eric Hachulla, Paloma Garcla De La Pena Lefebvre, Massimiliano Limonta, Petros Sfikakis, Maurizio Cutolo, Lidia P. Ananieva, Laszlo Czirjak, Christopher Denton, Giacomo De Luca, Lorenzo Dagna

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

9 Scopus citations

Abstract

Objective: Interstitial lung disease (ILD) is the leading cause of morbidity and mortality in systemic sclerosis (SSc) patients. We aimed to investigate the impact of sex on SSc-ILD. Methods: EUSTAR SSc patients with radiologically confirmed ILD and available percentage predicted forced vital capacity (%pFVC) were included. Demographics and disease features were recorded. A change in %pFVC over 12 months (s.d. 6) (cohort 1) was classified into stable (≤4%), mild (5-9%) and large progression (≥10%). In those with 2-year longitudinal %pFVC (cohort 2), the %pFVC change at each 12-month (s.d. 6) interval was calculated. Logistic regression analyses [odds ratio (OR) and 95% CI] and Cox proportional hazards models adjusted for age and %pFVC were applied. Results: A total of 1136 male and 5253 female SSc-ILD patients were identified. Males were significantly younger, had a shorter disease duration, had a higher prevalence of CRP elevation and frequently had diffuse cutaneous involvement. In cohort 1 (1655 females and 390 males), a higher percentage of males had stable ILD (74.4% vs 69.4%, P = 0.056). In multivariable analysis, disease duration and %pFVC [OR 0.99 (95% CI 0.98, 0.99) and OR 0.97 (95% CI 0.95, 0.99), respectively] in males and age, %pFVC and anti-centromere [OR 1.02 (95% CI 1.00, 1.04), OR 0.97 (95% CI 0.96, 0.98) and OR 0.39 (95% CI 0.245, 0.63), respectively] in females were associated with large progression. The 1-year mortality rate was higher in males (5.1% vs 2.5%, P = 0.013). In cohort 2 (849 females and 209 males), a higher percentage of females showed periods of large progression (11.7% vs 7.7%, P = 0.023), the percentage of patients with none, one or two periods of worsening was not different. The overall death rate was 30.9% for males and 20.4% in females (P < 0.001). In the survival analysis, male sex was a predictor of mortality [OR 1.95 (95% CI 1.66, 2.28)]. Conclusions: Male SSc-ILD patients have a poorer prognosis and sex-specific predictors exist in SSc-ILD.

Original languageEnglish
Pages (from-to)2483-2491
Number of pages9
JournalRheumatology
Volume62
Issue number7
DOIs
StatePublished - 1 Jul 2023

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