Abstract
Systemic sclerosis (SSc) is a severe, chronic disease characterised by small vessel vasculopathy, autoantibodies production, and fibroblast dysfunction leading to an excessive deposition of collagen in the skin and internal organs. The beneficial effects of iloprost in improving symptoms of ischemia such as Raynaud's phenomenon (RP) and digital ulcers (DUs) in patients with SSc are largely due to modulating the disordered microcirculation. Literature data show that the long-term IV iloprost administration maintains efficacy in the treatment of vasculopathy, representing a rational therapeutic approach, since Raynaud's phenomenon and digital ulcers are two of the major causes of pain and disability in scleroderma patients. Intravenous iloprost may also play a role in promoting a favourable disease course, as a stabilization of cardio-pulmonary were observed in long-term studies. Current evidences are encouraging, but further randomized and controlled trials are needed to confirm these results.
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CITATION STYLE
Visalli, E., Amato, G., Gangi, M., Benenati, A., Cino, N., Gagliano, C., … Foti, R. (2017). Treatment with intravenous iloprost in patients with systemic sclerosis: A short review. Journal of Rare Diseases Research & Treatment, 2(4), 6–13. https://doi.org/10.29245/2572-9411/2017/4.1114
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