This protocol applies to patients with systemic sclerosis (SSc) who have developed interstitial lung disease (ILD) — a recognised pulmonary complication of SSc — and are not currently receiving immunosuppressive treatment. It does not apply to early inflammatory diffuse cutaneous SSc.
Interstitial lung disease is a serious and potentially progressive complication in systemic sclerosis. When ILD is present and no immunosuppressive therapy is in use, evidence-based intervention is indicated to address the risk of ongoing lung function decline.
The management of SSc-ILD in this setting involves immunosuppressive therapy. The evidence supports specific agents with defined treatment courses — the complete regimen, including options and their respective durations, is available in the full structured protocol.
The primary treatment target is improvement in percentage predicted forced vital capacity (FVC) at 24 months — a key measure of lung function stabilisation in SSc-ILD.
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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