Interstitial lung disease in lcSSc: what to do when nintedanib has not reduced the rate of FVC decline

This protocol addresses patients with interstitial lung disease (ILD) complicating limited cutaneous systemic sclerosis who are on mycophenolate mofetil and whose disease continues to progress despite a prior course of nintedanib — specifically when the target of reducing the annual rate of forced vital capacity decline was not achieved.

Clinical Scenario

Interstitial lung disease in limited cutaneous systemic sclerosis, currently on mycophenolate mofetil (MMF). Nintedanib — with or without continuing MMF — was the previous treatment step, targeting a meaningful reduction in the annual rate of FVC decline. That goal was not met, indicating progressive fibrosing SSc-ILD requiring escalation.

Previous Line — Failure Condition

The prior regimen included nintedanib, added with or without continuation of mycophenolate mofetil. The intended target — reduction in the annual rate of decline in forced vital capacity — was not achieved, defining the threshold for moving to the next treatment line.

Next-Line Treatment Approach (Partial)

For progressive fibrosing SSc-ILD that has not responded to the previous line, rituximab is among the agents recommended for consideration by clinical task forces. The complete protocol specifies the administration schedule, eligibility criteria, and monitoring plan — these details are available via the full regimen below.

Treatment Goal

Improvement or stabilization of percentage predicted forced vital capacity at 24 weeks.

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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