Systemic Sclerosis: Molecular Research and Novel Therapy
A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".
Deadline for manuscript submissions: closed (20 August 2024) | Viewed by 454
Special Issue Editor
Special Issue Information
Dear Colleagues,
Systemic sclerosis (SSc) is a rare chronic autoimmune connective tissue disease characterized by diffuse fibrosis of the skin and internal organs and vascular abnormalities, with a prevalence of 1.47 million people worldwide (https://doi.org/10.1016/j.clim.2023.109267). The mortality rate for this disease is higher than that of other rheumatic diseases, with no cure or drug that can stop progression. Like other autoimmune diseases, systemic sclerosis occurs more frequently in women, with a peak of onset during the fifth decade of life. SSc is a rare disease and there is great heterogeneity between patients in terms of the onset, progression and characteristics. This makes diagnosis and targeted treatment even more difficult. Pathogenesis is dominated by vascular changes, autoimmunity with distinct autoantibodies and the activation of both innate and adaptive immunity. The cause of systemic sclerosis is still unknown. However, it is believed that during the early stages of the disease, an over-reactive immune system causes systemic inflammation and injury to the endothelial cells of blood vessels. The unstoppable progression of vascular and fibrotic organ damage accounts for the chronic morbidity and ensuing high mortality.
The management of SSc has improved and there is an increasing number of randomized clinical trials, with new triggers, molecular pathways and drugs emerging. Data from these trials suggest a significant potential for several new therapeutic options for SSc in the near future. Combining drugs with immunomodulatory, vascular remodeling and antifibrotic properties to address the three main features that lead to sustained disease exacerbation may be an option.
This Special Issue is dedicated to molecular research and novel therapy necessary for attaining better knowledge about the diagnosis, prognosis and treatment of SSc.
Dr. Carole Nicco
Guest Editor
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Keywords
- systemic sclerosis
- rare disease
- T cells
- interstitial lung disease
- autoimmunity
- fibrosis
- connectivitis
- diffuse cutaneous systemic sclerosis
- interstitial lung disease
- pulmonary function test
- scleroderma
- digital ulcers
- myocardial fibrosis
- interstitial fibrosis
- rheumatoid lung disease
- systemic disease
- primary biliary cholangitis
- Raynaud syndrome
- biomarkers
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