Emerging treatments for scleroderma/systemic sclerosis

Area of Science:

• Rheumatology
• Dermatology
• Immunology

Background:

• Systemic sclerosis (SSc) is a complex connective tissue disease causing progressive fibrosis in skin and organs.
• Managing SSc, particularly cutaneous manifestations, presents significant clinical challenges, often influenced by extracutaneous involvement.

Purpose of the Study:

• To review current and emerging therapeutic strategies for managing systemic sclerosis (SSc).
• To outline evidence-based recommendations for treating SSc, considering diverse clinical presentations and organ involvement.

Main Methods:

• Review of current literature on SSc management.
• Analysis of first- and second-line treatment options based on SSc subtypes and manifestations.
• Consideration of emerging therapies and supportive care strategies.

Main Results:

• Methotrexate is a common first-line therapy for early progressive cutaneous SSc.
• Treatment selection varies: methotrexate/rituximab for diffuse skin disease with arthritis, methotrexate/IVIG for myositis, and mycophenolate mofetil/rituximab for cutaneous and lung disease.
• Second-line options include phototherapy, IVIG, or rituximab for refractory cases. Emerging therapies like abatacept and tocilizumab are under investigation.
• Supportive care for pruritus, telangiectasias, calcinosis cutis, microstomia, and Raynaud's phenomenon is essential.

Conclusions:

• Systemic sclerosis is a heterogeneous disease requiring individualized, evidence-based treatment plans.
• Assessment of extracutaneous involvement is crucial for selecting appropriate therapies.
• Ongoing research into novel treatments offers hope for improved SSc management.