Systemic sclerosis in adults. Part I: Clinical features and pathogenesis

Area of Science:

• Rheumatology
• Immunology
• Dermatology

Background:

• Systemic sclerosis (SSc) is a rare, complex autoimmune connective tissue disease.
• It is characterized by progressive skin fibrosis, vasculopathy, immune dysregulation, and genetic factors.
• Current classification relies on clinical subtypes, but autoantibody profiles offer new insights.

Purpose of the Study:

• To review the epidemiology, clinical characteristics, and pathogenesis of SSc in adults.
• To emphasize the role of skin manifestations in early SSc detection and management.
• To highlight the importance of dermatologists in diagnosing and managing SSc.

Main Methods:

• Review of current literature on SSc epidemiology, pathogenesis, and classification.
• Analysis of the significance of autoantibody profiles in predicting disease course and organ involvement.
• Emphasis on clinical presentation, particularly cutaneous manifestations.

Main Results:

• Autoantibody profiles are predictive of skin and internal organ involvement and disease course.
• Cutaneous manifestations are often early signs of SSc progression and activity.
• Dermatologists play a key role in recognizing early signs and managing SSc.

Conclusions:

• Reconsideration of SSc classification based on autoantibody profiles is warranted.
• Early diagnosis and treatment initiation in SSc can significantly improve patient outcomes.
• Dermatologists are essential in the early identification and management of systemic sclerosis.