Interstitial Lung Disease in Systemic Sclerosis: What to Do When Nintedanib Has Not Slowed FVC Decline

This protocol addresses patients with systemic sclerosis‑associated interstitial lung disease (SSc‑ILD), currently on mycophenolate mofetil, where prior escalation to nintedanib has not achieved a reduction in the annual rate of forced vital capacity decline.

Clinical Scenario

Systemic sclerosis with interstitial lung disease (SSc‑ILD), ongoing mycophenolate mofetil therapy, and progressive fibrosing disease despite prior treatment escalation.

Previous Line — Target Not Achieved

Nintedanib was added (with or without continuation of mycophenolate mofetil) as the prior escalation step, targeting a reduction in the annual rate of FVC decline. This protocol defines the next step when that goal is not met.

Next-Line Approach (partial)

For progressive fibrosing SSc‑ILD, the next step involves rituximab, a targeted biologic agent given as a structured course. The treatment goal is improvement or stabilization of percentage predicted forced vital capacity at 24 weeks. The complete regimen and sequencing are available in the full protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1136/ard-2024-226430

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

The task force recommended that rituximab should be considered for the treatment of SSc-ILD.

The predicted FVC at 24 weeks compared with baseline was significantly improved in the rituximab group compared with the placebo group (0.09% vs -2.87%; difference 2.96% (95% CI 0.08% to 5.84%); p=0.044).

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