Limited cutaneous systemic sclerosis
ICD-10 M34.1 · ICD-11 4A42.2

Interstitial Lung Disease in Limited Cutaneous Systemic Sclerosis When First-Line Immunosuppression Has Not Achieved the Forced Vital Capacity Target

Clinical Scenario

This protocol covers patients with limited cutaneous systemic sclerosis complicated by interstitial lung disease (ILD) who are not currently on immunosuppressive treatment — specifically those who have completed first-line immunosuppression without achieving the expected improvement in lung function.

First-Line Treatment — Failure Condition

First-line therapy for SSc-ILD — using mycophenolate mofetil, oral cyclophosphamide, or intravenous rituximab — did not achieve the goal of improvement in percentage predicted forced vital capacity at 24 months.

This protocol defines the next step to take after that failure.

Next-Line Approach

The regimen for this situation centres on an antifibrotic agent targeting progressive fibrosing ILD. It can be used as monotherapy or in combination with an immunosuppressive agent, depending on the clinical picture.

Complete regimen, sequencing, and decision criteria are in the full protocol ↓

Treatment goal: Reduction in the annual rate of decline in forced vital capacity.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1136/ard-2024-226430

MMF (1A), cyclophosphamide (1A) or rituximab (1A) should be considered for the treatment of SSc-ILD.

Nintedanib should be considered alone or in combination with MMF for the treatment of SSc-ILD.

In SENSCIS, 576 SSc-ILD patients were randomly assigned to receive 150 mg of nintedanib, administered orally twice daily or placebo.

In the primary endpoint analysis, the adjusted annual rate of change in FVC was -52.4 mL per year in the nintedanib group and -93.3 mL per year in the placebo group.

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