Sex influence on outcomes of patients with systemic sclerosis–associated interstitial lung disease: a EUSTAR database analysis
Authors: Campochiaro Corrado , Hoffmann-Vold Anna-Maria , Avouac Jerome , Henes Jörg , de Vries-Bouwstra Jeska , Smith Vanessa , Siegert Elise , Airò Paolo , Oksel Fahrettin , Pellerito Raffaele , Vanthuyne Marie , Pozzi Maria Rosa , Inanc Murat , Sibilia Jean , Gabrielli Armando , Distler Oliver , Allanore Yannick ,   EUSTAR Collaborators, Cerinic Marco Matucci , Walker Ulrich , Iannone Florenzo , Becvar Radim , Cuomo Giovanna , Montecucco C , Carreira Patricia E , Iudici Michele , Kucharz Eugene J , Zanatta Elisabetta , Bancel Pr Dominique Farge , Hesselstrand Roger , Balbir-Gurman Alexandra , Pellerito Raffaele , Bertoldo Eugenia , Damjanov Nemanja , Granollers Vera Ortiz-Santamaria , Heitmann Stefan , Salvador Maria João , Stamenkovic Bojana , Selmi Carlo Francesco , Herrick Ariane , ller-Ladner Ulf Mü , Engelhart Merete , Riccieri Valeria , Ionescu Ruxandra Maria , Gheorghiu Ana Maria , Sunderkötter Cord , Distler Jörg , Ingegnoli Francesca , Mouthon Luc , Cantatore Francesco Paolo , Ullman Susanne , Wiland Piotr , Vanthuyne Marie , Saar Petra , Herrmann Kristine , De Langhe Ellen , Mayer Miroslav , Yavuz Sule , de Souza Müller Carolina , Zenone Thierry , Vacca Alessandra , Solanki Kamal , Rosato Edoardo , Yargucu Fahrettin Oksel Figen , Tanaseanu Cristina-Mihaela , Foti Rosario , Furst Daniel E , Adler Peter Villiger Sabine , Martín Jorge Juan González , Litinsky Ira , Del Galdo Francesco , Seskute Goda , Saketkoo Lesley Ann , Kerzberg Eduardo , Castellví Ivan , Spertini François , Hsu Vivien M , Martin Thierry , Schmeiser Tim , Majewski Dominik , Bernardino Vera , Puttini Piercarlo Sarzi , Moroncini Gianluca , Stork Jiri , Hachulla Eric , de la Pena Lefebvre Paloma Garcıa , Limonta Massimiliano , Sfikakis Petros , Cutolo Maurizio , Ananieva Lidia P , Czirjak Laszlo , Denton Christopher , De Luca Giacomo , Dagna Lorenzo 
Published: November 2022

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 &lt; 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.