摘要
混合型低血球蛋白血症(MCs)是一种系统性血管炎包括皮肤、关节、周围神经和几种内脏器官。肝炎C病毒(HCV)被认为是大多数MC患者以及大量自身免疫、淋巴增生以及肿瘤性紊乱的病因。本文,HCV相关的MC是一种重要的在人体的一种病毒感染的自身免疫或者肿瘤性疾病的模型。关于MC的治疗性策略,我们能够通过病因(抗病毒)、发病、症状药物(主要为免疫抑制、皮质类固醇、血浆置换)手段治疗不同时期的患者。在大多人个人患者中,MC显示轻度、缓慢进展的临床病程只需要症状治疗,通常低剂量的皮质类固醇。考虑到MC的发病机制,根除HCV应被视为治疗MC的黄金标准。组合的peg-干扰素-α/利巴韦林或新的抗病毒药物的使用可能导致在很可能缓解MC的病例中有大量HCV的根除。另一方面,主要基于环磷酰胺或利妥昔单抗,高剂量皮质类固醇和血浆aphaeresis的侵袭性免疫抑制和抗炎治疗可以成功地治疗具有多器官受累的快速进行性、弥漫性血管炎的存在。此外,顺序/组合抗病毒或者免疫抑制治疗能够可以代表一种有用的治疗策略,特别是在具有主要临床表现的MCs患者中。应根据临床情况的严重程度为每名患者决定MC的治疗。因此,需要认真对待疾病特别注意癌症发病的可能性,如细胞淋巴瘤。本综述着重于MCs患者的不同治疗策略,包括治疗冷球蛋白血症皮肤溃疡,代表了该疾病最令人不舒服的并发症之一。
关键词: 肝炎C病毒,混合型低血球蛋白血症,冷血球血管炎,淋巴瘤,皮肤溃疡,利妥昔单抗
图形摘要
Current Drug Targets
Title:Treatment of HCV-Related Mixed Cryoglobulinemia
Volume: 18 Issue: 7
关键词: 肝炎C病毒,混合型低血球蛋白血症,冷血球血管炎,淋巴瘤,皮肤溃疡,利妥昔单抗
摘要: Mixed cryoglobulinemia syndrome (MCs) is a systemic vasculitis, involving skin, joints, peripheral nerves, and several internal organs. Hepatitis C virus (HCV) is recognized as the etiologic agent for the majority of MCs patients, as well as of number of autoimmune, lymphoproliferative, and neoplastic disorders. In this context, HCV-related MCs represents an important model autoimmune/ neoplastic disease triggered by a virus in humans. With regard the therapeutic strategies of MCs, we can treat these patients at different steps by means of etiological (antivirals), pathogenetic, symptomatic drugs (mainly immunosuppressors, corticosteroids, plasmapheresis). In the majority of individuals, MCs shows a mild, slow-progressive clinical course needing only symptomatic treatments, generally low doses of corticosteroids. Considering the etiopathogenesis of MCs, the eradication of HCV should be considered the gold standard in the treatment of MCs. The use of combined peg-interferon- α/ribavirin and/or novel antiviral drugs may lead to HCV eradication in a significant percentage of cases with possible remission of MCs. On the other hand, the presence of rapidly progressive, diffuse vasculitis with multiple organ involvement may be successfully treated with aggressive immunosuppressive and anti-inflammatory therapies, mainly based on cyclophosphamide or rituximab, high dose corticosteroids, and plasma aphaeresis. Moreover, sequential/combined antiviral or immunosuppressive treatments could represent an useful therapeutic strategy particularly in MCs patients with major clinical manifestations.
The treatment of MCs should be decided for every patient according to the severity of clinical picture. Thus, a careful follow-up of the disease is necessary, with particular attention to the possibility of cancers onset, such as B-cell lymphoma. The present review focuses on the different therapeutic strategies in patients with MCs, including the treatment of cryoglobulinemic skin ulcers, which represents one of the most discouraging complications of the disease.Export Options
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Cite this article as:
Treatment of HCV-Related Mixed Cryoglobulinemia, Current Drug Targets 2017; 18 (7) . https://dx.doi.org/10.2174/1389450116666150825112105
DOI https://dx.doi.org/10.2174/1389450116666150825112105 |
Print ISSN 1389-4501 |
Publisher Name Bentham Science Publisher |
Online ISSN 1873-5592 |
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