Kastriot Kastrati, Marco Sprecher, Florine Weber, Emily Langballe, Daniel Molnar, Lena Theresa Göthans, Karolina Anderle, Håvard Fretheim, Hilde Jenssen Bjørkekjær, Phuong Phuong Diep, Marthe Mæhlen, Daniel Mrak, Nadège Rempp, Bojana Müller-Durovic, Cathrine Brunborg, Ana Vieira, Ana Cristina Correia, Coralie Bouillot, Cosimo Bruni, Christian Clarenbach, Thomas Frauenfelder, Trond Mogens Aaløkken, Natasha Moe, Helmut Prosch, Øyvind Midtvedt, Helena Andersson, Paul Studenic, Øyvind Molberg, Daniel Aletaha, Oliver Distler, Helga Lechner-Radner, Anna-Maria Hoffmann-Vold
Management of SjD-ILD has evolved towards broader and more consistent use of immunosuppressive therapy. However, a substantial proportion of patients remained untreated, and treatment was more frequently initiated in patients with greater symptom burden and impaired lung function, which emphasises the need for earlier and evidence-based intervention strategies.
BACKGROUND: Interstitial lung disease in Sjögren disease (SjD-ILD) is a clinically relevant and potentially progressive complication, yet treatment remains empirical and poorly supported by evidence.
METHODS: This multicentre observational study across three European expert centres (Oslo, Zurich, and Vienna) included patients with SjD-ILD who were diagnosed between 1997 and 2025, fulfilled the 2016 ACR/EULAR SjD criteria and had ILD confirmed by high-resolution computed tomography (HRCT). Treatment patterns and pulmonary function were analysed across four predefined calendar periods (≤2006, 2007-2011, 2012-2016, ≥2017). Outcomes included therapeutic patterns, factors associated with treatment, longitudinal changes in pulmonary function and mortality. ILD progression and improvement were defined as absolute forced vital capacity (FVC) change (≥5% or ≥10%) over 12 ± 3 and 24 ± 3 months, and by 5-year all-cause mortality. Comparisons across periods used trend tests, and logistic regression was applied to identify factors associated with treatment. We involved people with lived experience in the study design and implementation.
RESULTS: Among 191 patients with SjD-ILD (mean age 59.9 ± 13.6 years, 81% females), 122 (63.9%) ever received immunosuppressive therapy, increasing from 52.4% before 2006 to 71.3% after 2017 (p = 0.03). Glucocorticoids were used in 81 patients (42.4%), rituximab in 48 (25.1%), azathioprine in 33 (17.3%), and mycophenolate-mofetil in 32 (16.8%). Patients with higher dyspnoea severity (OR 3.34, 95% CI 1.43-7.80) and FVC<70% predicted (OR 3.48, 95% CI 1.54-7.90) were more likely to be treated. Lymphocytic interstitial pneumonia was treated less frequently than non-specific interstitial pneumonia (OR 0.40, 95% CI 0.18-0.90). Over time, ILD progression remained largely unchanged, while ILD improvement (≥5% FVC increase) increased from 2.9% to 11.9% (p = 0.03). Over a mean follow-up of five years, 9 patients (4.8%) died, with no significant changes in mortality rates over time.
CONCLUSION: Management of SjD-ILD has evolved towards broader and more consistent use of immunosuppressive therapy. However, a substantial proportion of patients remained untreated, and treatment was more frequently initiated in patients with greater symptom burden and impaired lung function, which emphasises the need for earlier and evidence-based intervention strategies.