Treatment of Limited Cutaneous Systemic Sclerosis with Interstitial Lung Disease
Interstitial lung disease (ILD) is a recognised complication of limited cutaneous systemic sclerosis (lcSSc). When ILD is present and no immunosuppressive treatment has been started, a structured first-line approach is indicated to preserve lung function.
Clinical scenario
Patients with limited cutaneous systemic sclerosis who have developed interstitial lung disease and are not currently receiving any immunosuppressive therapy. This treatment-naive status means first-line options can be applied in full — the specific regimen is detailed in the full protocol.
Treatment approach
Immunosuppressive therapy is the cornerstone of management for SSc-ILD in this setting. Multiple evidence-based agents have been evaluated in controlled trials; the choice among them and the course structure are addressed in the full protocol.
Treatment goal
The primary clinical target is improvement in percentage predicted forced vital capacity (FVC) at 24 months, reflecting stabilisation or recovery of pulmonary function over the treatment course.
References
DOI: 10.1136/ard-2024-226430
MMF (1A), cyclophosphamide (1A) or rituximab (1A) should be considered for the treatment of SSc-ILD.
The SLS II compared a continuous 24-month course of MMF to a 12-month course of oral cyclophosphamide (followed by 12 months of placebo) in an RCT of SSc-ILD patients.
Each treatment group showed significant improvement in % predicted FVC at 24 months, 2.19% (95% CI 0.53% to 3.84%) for the MMF group and 2.88% (95% CI 1.19% to 4.58%) for the cyclophosphamide group.
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