Use of Extracorporeal Photopheresis in Scleroderma: A Review

Dermatology : International Journal for Clinical and Investigative Dermatology
Amy X DuRobert Gniadecki

Abstract

Scleroderma is a heterogeneous group of diseases that can be localized or systemic. Localized scleroderma is a fibrosis of the skin characterized by inflammation and thickening due to excessive collagen deposition, and systemic sclerosis (SSc) is characterized by vasculopathy, immune dysregulation and skin fibrosis. In general, the prognosis of scleroderma highly depends on the degree of visceral involvement and relates to the degree of skin fibrosis. Despite the numerous therapies used for patients with scleroderma, the disease-related morbidity and mortality are high. Studies have explored the effects of extracorporeal photopheresis (ECP) in scleroderma treatment. Originally used in the treatment of cutaneous T-cell lymphoma, ECP is an immunomodulatory procedure in which a patient's white blood cells are treated with 8-methoxypsoralen and exposed to UVA radiation to inhibit cell proliferation and induce immunosuppression. Multiple lines of evidence suggest that ECP may be a safe and possibly effective therapy for patients with scleroderma, specifically demonstrating improvement in patients with cutaneous manifestations of the disease. However, future studies assessing its role in managing visceral involvement are needed. Our ...Continue Reading

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Citations

Feb 9, 2020·Molecules : a Journal of Synthetic Chemistry and Natural Product Chemistry·Cristian ScheauAna Caruntu
Apr 29, 2021·Molecular Biology Reports·Newman OsafoAaron O Antwi
May 5, 2021·The Journal of Dermatological Treatment·Thilo GambichlerLaura Susok

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