Background/Purpose: The therapeutic landscape for systemic sclerosis- interstitial lung disease (SSc-ILD) has rapidly evolved over the past decade, with increasing adoption of immunosuppressive (IST) and anti-fibrotic therapies. However, real-world data on current treatment strategies, including combination regimens, treatment switches, and clinical predictors for treatment decisions, remain limited. This study aims to characterize treatment patterns and their clinical drivers in the contemporary era using the EUSTAR database.

Methods: We analyzed SSc-ILD patients from the EUSTAR registry first assessed since ³ 2017. Treatment courses were defined as any use of IST and/or anti-fibrotic drugs ³3 months. Treatment strategies were classified as: (1) switch: change from one therapy to another during follow-up; (2) discontinuation: stopping a primary therapy without initiating another; (3) unmodified: no changes during follow-up; (4) combination: concurrent use of ³ 2 drugs. Frequency and combinations of therapies were described. Logistic regression analyses were used to identify clinical factors associated with treatment strategies.

Results: Among 277 patients, 159 (57%) received IST and/or anti-fibrotic therapy since their first evaluation, Table 1. Multivariable analysis showed that shorter disease duration (OR 0.991, 95% CI 0.987–0.996, p< 0.001) and presence of myositis (OR 9.93, 95% CI 1.94–51.76, p=0.006) were associated with treatment initiation. The different treatment courses are summarized in Figure 1 and grouped according to the 4 most used IST: mycophenolate mofetil (total courses 377, panel A), methotrexate (total courses 187, panel B), rituximab (total courses 257, panel C) and tocilizumab (total courses 122, panel D). A total of 213 treatment switches occurred (see Figure 2). Higher mRSS (OR 1.031, 95% CI 1.002 – 1.062, p = 0.035) and arthritis (OR 3.034, 95% CI 1.551 – 5.936, p = 0.001) were associated with treatment switching in multivariable logistic regression. Younger age (OR 0.968, 95% CI 0.953 – 0.985, p < 0.001), higher dyspnea class (OR 1.557, 95% CI 1.189 – 2.039, p = 0.001), and inflammatory arthritis (OR 2.560, 95% CI 1.354 – 4.840, p = 0.004) were associated with combination therapy. No predictor was associated with treatment discontinuation.

Conclusion: In current clinical practice, a considerable amount of SSc-ILD patients is treated. Treatment decisions are influenced not only by lung function but also by systemic features such as arthritis and myositis. Combination strategies and treatment modifications are frequently used in SSc-ILD, reflecting a more individualized approach. These findings have important implications for future trial design and highlight the urgent need for evidence-based guidance on combination and sequential therapies.

Table 1. Demographic and clinical features of SSc-ILD patients treated at first-time assessment in year ≥ 2017.

Figure 1. Treatment courses according to the DMARD-based regimen (panel A mycophenolate mofetil-based courses; panel B methotrexate-based courses; panel C rituximab-based courses and panel D tocilizumab-based courses).

Figure 2. The use of csDMARD and bDMARDs in SSc-ILD patients switching therapies.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/current-treatment-strategies-in-systemic-sclerosis-interstitial-lung-disease-patients-real-world-insights-from-the-eustar-cohort-cp138/