[Scleroderma: an update on the pathogenesis and treatment]

Area of Science:

• Rheumatology and Immunology
• Dermatology
• Vascular Biology

Context:

• Scleroderma is a multisystemic disorder characterized by inflammation, vascular abnormalities, and fibrosis affecting the skin and internal organs.
• It is understood as a tripartite disease involving autoimmune, fibroblast, and endothelial defects, influenced by genetic, environmental, and infectious factors.
• The condition presents in systemic and localized forms, with Raynaud phenomenon affecting 90% of patients, indicating microcirculation compromise and capillary loss.

Purpose:

• To review the multifaceted nature of scleroderma, including its underlying pathophysiology, clinical manifestations, and current therapeutic limitations.
• To highlight the significance of microcirculation abnormalities, such as nail bed capillary malformations, in assessing disease severity and internal organ involvement.
• To evaluate the utility of the modified Rodnan skin score (MRSS-51) in predicting patient survival and assessing skin involvement, while clarifying its limitations as an activity measure.

Summary:

• Scleroderma involves inflammatory, vascular, and sclerotic changes across multiple organs, stemming from a combination of genetic, environmental, and infectious triggers.
• Raynaud phenomenon and nail bed capillary abnormalities are common, correlating with disease severity and internal organ compromise.
• The MRSS-51 is a valuable tool for assessing skin involvement and predicting survival but is not suitable for measuring disease activity or treatment efficacy.

Impact:

• This review provides a comprehensive overview of scleroderma, aiding clinicians in understanding disease complexity and patient prognosis.
• It emphasizes the importance of microvascular assessment and the MRSS-51 in managing scleroderma patients.
• The findings underscore the need for improved therapeutic strategies targeting the cutaneous manifestations of scleroderma.