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  • Systemic Sclerosis

    An Introduction to Systemic Sclerosis

    Systemic sclerosis (SSc) is a rare, life-threatening, rheumatologic autoimmune disease characterised by fibrosis of the skin and multiple organ targets. Treatment strategies involve immunosuppressive and antifibrotic drugs but their efficacy is modest. Haematopoietic stem-cell transplantation reduces long-term mortality, but confers the risk of treatment-related mortality. Nintedanib has been approved to slow the rate of decline in pulmonary function in adults with interstitial lung disease associated with SSc, but does not affect skin fibrosis. Many novel therapeutic targets are emerging, including interleukin (IL) -6 (tocilizumab), IL-4 and IL-13, B cell pathology, and the Janus kinase (JAK)-signal transducer and activator of transcription (STAT) pathway. However, there remains no approved targeted therapies.

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