2013-2018年在皮肤性病学诊所治疗的自身免疫性天疱疮(AP)患者的治疗方式和病程

Sucro Madzgalj, Dragana Jakovljević, D. Živanović
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摘要

自身免疫性天疱疮(AP)是最严重的皮肤病之一。AP属于器官特异性自身免疫性疾病,其自身抗体针对角质形成细胞上的细胞表面抗原,主要是粘粒。根据临床和组织病理学特征以及不同的靶抗原,已经描述了三种主要类型的AP:寻常型天疱疮(PV),叶状天疱疮(PF),每种都有自己的亚型和副肿瘤性天疱疮(PNP)。AP的临床病程和预后取决于其亚型、治疗方式及其副作用。治疗的主要目标是在没有任何全身治疗或最小治疗剂量(“维持治疗”)的情况下实现和维持缓解。目的:回顾性分析各种形式的AP住院患者,分析其临床病程并评价其治疗方法。材料和方法:本研究共纳入2013 - 2018年塞尔维亚临床中心皮肤性病学诊所收治的158例确诊AP住院患者,随访6 - 12个月。采用描述性统计和分析性统计对人口学和临床资料以及治疗方法进行分析。结果:最常见的亚型为PV (72.15%);发病年龄在39-59岁之间的患者最多(46.84%)。常规皮质类固醇治疗最常见(67.41%),硫唑嘌呤(66.86%)是最常见的免疫抑制药物。60.8%的患者无不良反应或疾病并发症。在随访期间,截至2018年底,48.1%的患者获得缓解。结论:我们发现PV的发病率很高。在我们的患者中使用的治疗方法是根据天疱疮亚型和病程。相当数量的患者达到了临床缓解。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Therapeutic modalities and course of the disease in patients with autoimune pemphigus (AP) treated at the Clinic of dermatovenerology in the period 2013-2018
Introduction: Autoimmune pemphigus (AP) is one of the most severe dermatologic diseases. AP belongs to the group of organ-specific autoimmune disorders, with autoantibodies directed against cellsurface antigens on keratinocytes, mostly desmogleins. Three main types of AP have been described, depending on the clinical and histopathological features and the different target antigens: Pemphigus vulgaris (PV), Pemphigus foliaceus (PF), each with its own subtypes and paraneoplastic pemphigus (PNP). The clinical course and prognosis of AP depend on its subtype, therapeutic modalities and their side effects. The main goal of the treatment is to achieve and maintain remission without any systemic therapy or with minimal therapeutic doses ("maintenance therapy"). Aim: A retrospective study of hospitalized patients with various forms of AP, analysis of clinical course and evaluation of therapeutic modalities employed. Material and methods: A total of 158 inpatients with diagnosed AP, admitted to the Clinic of Dermatovenereology, Clinical Centre of Serbia, from 2013 to 2018, with a subsequent followup of 6 to 12 months, were included in this study. Demographic and clinical data as well as treatment approaches were analyzed using descriptive and analytic statistics. Results: The most common subtype encountered was PV (72.15%); in the largest number of patients (46.84%) the disease occurred between ages 39-59. Conventional corticosteroid treatment was most frequently used (67.41%), along with azathioprine (66.86%) as the most common immunosuppressive drug. In 60.8% of patients no side effects or disease complications were recorded. During the follow-up, at the end of 2018, 48.1% of patients achieved remission. Conclusion: We have shown a high incidence of PV. Treatment approaches used in our patients were in accordance with the pemphigus subtype and course of the disease. A significant number of patients achieved clinical remission.
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