Management of systemic sclerosis: the first five years

Area of Science:

• Rheumatology
• Immunology
• Pulmonology

Background:

• Systemic sclerosis (SSc) is a heterogeneous autoimmune disease with variable clinical courses.
• Cardiopulmonary complications are the leading cause of mortality in SSc patients.
• Early risk stratification is crucial for effective management.

Purpose of the Study:

• To provide a risk-stratified, evidence-based management approach for SSc patients.
• To focus on patients within the first five years of disease onset.
• To stratify management based on prognostic factors like skin involvement and autoantibody status.

Main Methods:

• Review of current literature and clinical guidelines.
• Risk stratification based on SSc patient subsets.
• Evidence-based recommendations for managing SSc-associated cardiopulmonary diseases.

Main Results:

• Management strategies for SSc-associated pulmonary arterial hypertension have improved with combination therapies.
• New evidence suggests early immunosuppression for SSc-associated interstitial lung disease (SSc-ILD) may be beneficial.
• An antifibrotic agent was approved in 2019 for SSc-ILD, and autologous hematopoietic stem cell transplant shows a mortality benefit in select cases.

Conclusions:

• Risk stratification by skin distribution and autoantibody status aids in managing SSc within the first five years.
• Treatment paradigms for SSc-ILD are evolving towards earlier intervention.
• Tailored management strategies are essential for improving outcomes in SSc patients.