Systemic Sclerosis with Interstitial Lung Disease — After Immunosuppressive Treatment Did Not Improve Lung Function
Clinical Scenario
This protocol addresses patients with interstitial lung disease complicating systemic sclerosis (SSc-ILD) who are not currently on immunosuppressive treatment and do not have early inflammatory diffuse cutaneous systemic sclerosis.
Prior Treatment — Failure Condition
The preceding treatment line used mycophenolate mofetil, oral cyclophosphamide, or rituximab. This protocol is indicated when that course did not achieve the expected improvement in percentage predicted forced vital capacity at 24 months.
Next-Line Approach (Partial Overview)
The protocol centres on an antifibrotic agent for progressive fibrosing SSc-ILD, which may be used alone or alongside another agent. The complete regimen, decision criteria, and full sequencing are available in the structured protocol.
Treatment Goal
The primary clinical objective is reduction in the annual rate of decline in forced vital capacity — slowing progressive loss of lung function in SSc-ILD.
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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