医学
尼罗替尼
达沙替尼
肾源性系统性纤维化
伊马替尼
纤维化
耐受性
甲磺酸伊马替尼
酪氨酸激酶
酪氨酸激酶抑制剂
结缔组织病
结缔组织
结缔组织病
内科学
药理学
癌症研究
病理
疾病
不利影响
自身免疫性疾病
受体
癌症
髓系白血病
肾脏疾病
作者
Jörg H. W. Distler,Oliver Distler
标识
DOI:10.1136/ard.2009.120196
摘要
Systemic sclerosis (SSc) is a fibrosing connective tissue disease with significantly increased mortality. Therapeutic options to treat fibrosis are limited. The small molecule tyrosine kinase inhibitor imatinib and related drugs such as dasatinib and nilotinib target simultaneously two of the major profibrotic pathways, TGFβ- and PDGF- signaling. Imatinib, dasatinib and nilotinib inhibit collagen synthesis in cultured fibroblasts and have potent anti-fibrotic effects in animal models of different fibrotic diseases. Moreover, several case reports, case series and uncontrolled studies on patients with SSc, mixed connective tissue disease, nephrogenic systemic fibrosis and in particular sclerodermatous graft versus host disease (cGvHD) report regression of fibrosis and good tolerability. However, the results of larger controlled trials, which are currently ongoing, are needed before any conclusions on efficacy and tolerability can be drawn. Until the results of these trials are available, we discourage off-label use of Imatinib in single patients.
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