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Systemic sclerosis: current pathogenetic concepts and future prospects for targeted therapy
Abstract:
Although there is no cure for systemic sclerosis, the disease is certainly treatable. And the more that is understood of its pathogenesis, the better the prospects for treatment. Excessive collagen deposition in the skin and viscera is responsible for the morbidity and mortality. The immune system plays a critical role in setting up the fibrotic process, but impaired regulation of collagen gene expression probably underlies the persistence of fibrosis. The development of effective antifibrotic drugs, in particular agents that inhibit transcription factors involved in the activation of type I collagen genes, is likely to be the way forward for treatment of the later stages of diffuse systemic sclerosis, with immunomodulatory therapy for the earlier stages.
Insights
Systemic sclerosis is treatable, with better prospects as pathogenesis is understood. Targeting collagen gene expression with antifibrotic drugs and using immunomodulatory therapy offers new treatment avenues.
Area of Science:
- Rheumatology
- Immunology
- Fibrosis Research
Background:
- Systemic sclerosis is a chronic autoimmune disease characterized by excessive collagen deposition.
- This fibrosis in skin and visceral organs leads to significant morbidity and mortality.
- The immune system initiates the fibrotic process, but dysregulated collagen gene expression sustains it.