Treatment of Skin Fibrosis in Limited Cutaneous Systemic Sclerosis

This protocol covers the management of skin fibrosis as it occurs in limited cutaneous systemic sclerosis — a specific, clinically meaningful manifestation of the disease that calls for a targeted, evidence-guided therapeutic approach.

Clinical Scenario

The therapeutic focus is skin fibrosis in the context of limited cutaneous systemic sclerosis. Accurate identification of this manifestation is essential, as it determines both the treatment strategy and the monitoring framework used to assess response.

Treatment Approach

The protocol designates specific pharmacological agents — each supported by distinct levels of clinical evidence — as the recommended strategy for addressing skin fibrosis. Which agents apply, and how they are selected and sequenced, is detailed in the full protocol.

Treatment Goal

The primary objective is improvement in modified Rodnan skin score (mRSS), a validated measure used to quantify the extent of skin involvement in systemic sclerosis.

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References

DOI: 10.1136/ard-2024-226430

Methotrexate (1B), mycophenolate mofetil (MMF) (1B) and/or rituximab (1A) should be considered for treatment of SSc skin fibrosis.

Patients received four intravenous doses of the assigned intervention (rituximab 375 mg/m2 or placebo; once per week for 4 weeks).

Post hoc analyses of SLS-II identified modified Rodnan skin score (mRSS) improvements from baseline to 24 months for both MMF and cyclophosphamide.

The absolute improvement in mRSS at 24 weeks was significantly higher in the rituximab group than in the placebo group.

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