先天性和获得性干扰素病变:干扰素治疗的不同方法

Nesterova Irina, S. Kovaleva, V. Malinovskaya, G. Chudilova, T. Rusinova
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引用次数: 1

摘要

本章回顾了各种干扰素(IFN)系统紊乱-干扰素病。作者描述了与ifn α -过表达相关的I型干扰素病的临床特点,这是一种罕见的孟德尔遗传疾病。某些自身免疫性疾病(系统性红斑狼疮(SLE)、血管炎、免疫失调综合征等)也以IFNα的过量产生为特征。此外,最常见的干扰素病变是IFN缺乏,儿童和成人先天性或获得性IFNα/IFNβ和IFNγ缺乏。IFNα/IFNβ缺乏与严重复发性病毒感染相关,IFNγ缺乏引起分枝杆菌感染。描述了干扰素矫正治疗方法。I型干扰素病变(生物制剂)的靶向治疗结合IFNα并使高水平的IFNα正常化。另一方面,先天性干扰素α缺乏的患者需要干扰素替代治疗。在获得性干扰素α缺乏的情况下,进行分化干扰素矫正治疗。在替代疗法和干扰素矫正疗法中,重组人IFNα2b联合抗氧化剂(Viferon®)均可使用,因为其应用安全,临床疗效好,无副作用。
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Congenital and Acquired Interferonopathies: Differentiated Approaches to Interferon Therapy
This chapter reviews various interferon (IFN) system disturbances—interferonopathies. The authors describe clinical specifics of type I interferonopathy associated with overexpression of IFNα—which is a rare Mendelian genetic disease. Certain autoimmune diseases (systemic lupus erythematosus (SLE), vasculitis, immune dysregulation syndrome, etc.) are also characterized by overproduction of IFNα. Furthermore the most common interferonopathies are described—deficiencies of IFN, congenital or acquired IFNα/IFNβ and IFNγ deficiencies in children and adults. Deficiency of IFNα/IFNβ associated with severe recurrent viral infections and deficiency of IFNγ cause mycobacterial infection. Interferon-corrective therapy methods are described. The target therapy of type I interferonopathies (biologics) binds IFNα and normalizes the high level of IFNα. From the other side, patients with congenital IFNα deficiencies are needed in replacement IFN therapy. In case of acquired IFNα deficiency, the differentiated interferon-corrective therapy is performed. In both replacement and interferon-corrective therapies, recombinant human IFNα2b in complex with antioxidants (Viferon®) can be used, because their application is safe and has good clinical efficiency and no side effects.
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