Systemic sclerosis
ICD-10 M34 · ICD-11 4A42

Treatment of Systemic Sclerosis with Interstitial Lung Disease in Early Inflammatory Diffuse Cutaneous SSc

This protocol targets a specific, clinically important subgroup: patients with interstitial lung disease (ILD) occurring in the setting of early inflammatory diffuse cutaneous systemic sclerosis, where preservation of lung function is the central therapeutic priority.

Clinical scenario

The protocol applies to patients with early inflammatory diffuse cutaneous systemic sclerosis who have developed interstitial lung disease. The concurrent presence of active cutaneous inflammation and pulmonary involvement defines this subgroup and shapes both the indication and the treatment selection covered here.

Treatment approach

The protocol addresses the use of tocilizumab for interstitial lung disease in early inflammatory systemic sclerosis. The full selection criteria, monitoring requirements, and dosing schedule are contained in the complete regimen.

The complete protocol — including specific eligibility criteria, the dosing algorithm, and monitoring plan — is available via the link below.

Primary treatment goal

Preservation of forced vital capacity (FVC) at 48 weeks, reflecting stabilisation of lung function as the key measure of therapeutic success in SSc-ILD.

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References

DOI: 10.1136/ard-2024-226430

Tocilizumab should be considered for the treatment of SSc-ILD.

Based on these data, the FDA approved the use of tocilizumab for the treatment of SSc-ILD.

In the phase 3 trial, the 48-week LSM change from baseline in FVC% predicted was -4.6 in the placebo group and -0.4 in the tocilizumab group (difference 4.2 (95% CI 2.0 to 6.4); nominal p=0.0002).

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