ILD in Systemic Sclerosis When First-Line Therapy Has Not Achieved Stabilisation

Clinical Scenario

This protocol applies to patients with systemic sclerosis (SSc) and concurrent interstitial lung disease (SSc-ILD) in whom an initial course of treatment has not met the primary pulmonary goals, and a next step in management is required.

When First-Line Treatment Falls Short

The preceding line of therapy for SSc-ILD — which may include mycophenolate mofetil, tocilizumab, nintedanib, cyclophosphamide, rituximab, or combination regimens — is considered insufficient when the following targets are not achieved:

Improvement or stabilisation of FVC%
Stabilisation or reduction of the extent of ILD on HRCT
Improvement of breathlessness and cough

Next-Line Approach — Partial Overview

When existing immunosuppressant therapy has proven insufficient, the next step involves adjusting or extending the immunosuppressant regimen. Where progressive pulmonary fibrosis has developed, an additional targeted agent may be incorporated. The full eligibility criteria, sequencing, and decision points are available in the complete protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1183/13993003.02533-2024

We recommend using tocilizumab in a subgroup (S, M) and suggest using MMF, rituximab (C, VL), and cyclophosphamide (C, L) in patients with SSc-ILD.

If on mono or combination treatment, add or switch immunosuppressant

If progressive pulmonary fibrosis, add nintedanib

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