Treatment of Early Diffuse Cutaneous Systemic Sclerosis with Poor Prognosis
Selected patients with early diffuse cutaneous systemic sclerosis (dcSSc) who carry poor prognostic features — and in whom advanced cardiorespiratory involvement is absent — represent a distinct clinical population for which a specific treatment protocol applies.
Clinical Scenario
The target population is patients with early dcSSc and poor prognosis. The absence of advanced cardiorespiratory involvement is a defining eligibility criterion: this qualification shapes both patient selection and the intensity of the approach undertaken.
Treatment Approach
This protocol employs high-intensity immunosuppression as the foundation of treatment — the complete regimen, including the subsequent steps and full patient-selection criteria, is available in the structured protocol.
Clinical Goal
The primary measure of treatment success is event-free survival at 72 months.
References
DOI: 10.1136/ard-2024-226430
High-intensity immunosuppression (usually including cyclophosphamide) followed by autologous HSCT may be considered for the treatment of selected patients with early dcSSc and poor prognosis, in the absence of advanced cardiorespiratory involvement.
The event-free survival analysis showed accordingly, that 74% of patients in the transplant arm remained event free at month 72 vs 47% of patients in the cyclophosphamide arm.
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