Suppr超能文献

天疱疮——新冠疫情期间临床、研究与治疗的关键

Pemphigus-The Crux of Clinics, Research, and Treatment during the COVID-19 Pandemic.

作者信息

Marinović Branka, Miše Joško, Jukić Ines Lakoš, Bukvić Mokos Zrinka

机构信息

Department of Dermatology and Venereology, University Hospital Centre Zagreb, School of Medicine University of Zagreb, Šalata 4, 10000 Zagreb, Croatia.

Department of Dermatology and Venereology, University Hospital Centre Zagreb, European Reference Network (ERN)-Skin Reference Centre, Kišpatićeva 12, 10000 Zagreb, Croatia.

出版信息

Biomedicines. 2021 Oct 28;9(11):1555. doi: 10.3390/biomedicines9111555.

Abstract

Pemphigus is a rare autoimmune disease characterised by the production of pathogenic autoantibodies in response to different desmosome proteins. The pathophysiological process leads to the development of blisters and erosions on mucosal and/or skin surfaces. The classical clinical variants of pemphigus are pemphigus vulgaris and pemphigus foliaceus. A diagnostic delay is very common in pemphigus, especially among patients with mucosal involvement. However, in recent years we have witnessed considerably fewer patients with extensive mucocutaneous manifestations, since patients with oral lesions are referred to dermatologists to start the treatment much sooner than they had been previously. Among non-classical variants of pemphigus, unusual cases with discrepancies between autoantibody profiles and clinics challenge the "desmoglein compensation theory". The identification of several other autoantigens that perform a role in the pathogenesis of different variants of pemphigus will progress immunodermatology towards an approach that will determine personalized pemphigus subtypes for each patient. Comorbidities among patients are primarily associated with the prolonged use of corticosteroids and other immunosuppressive agents. The SARS-CoV-2 pandemic raised concerns regarding the immunosuppressive effects of treatment and the risk of a more complicated COVID-19 infection, as well as on the ability to develop an adequate vaccine response.

摘要

天疱疮是一种罕见的自身免疫性疾病,其特征是针对不同的桥粒蛋白产生致病性自身抗体。病理生理过程导致黏膜和/或皮肤表面出现水疱和糜烂。天疱疮的经典临床变型是寻常型天疱疮和落叶型天疱疮。天疱疮的诊断延迟非常常见,尤其是在有黏膜受累的患者中。然而,近年来我们发现有广泛黏膜皮肤表现的患者明显减少,因为有口腔病变的患者比以前更早被转诊至皮肤科医生处开始治疗。在天疱疮的非经典变型中,自身抗体谱与临床情况不符的不寻常病例对“桥粒芯糖蛋白补偿理论”提出了挑战。确定在不同变型天疱疮发病机制中起作用的其他几种自身抗原,将推动免疫皮肤病学朝着为每位患者确定个性化天疱疮亚型的方向发展。患者的合并症主要与长期使用皮质类固醇和其他免疫抑制剂有关。严重急性呼吸综合征冠状病毒2(SARS-CoV-2)大流行引发了人们对治疗的免疫抑制作用、更复杂的2019冠状病毒病(COVID-19)感染风险以及产生充分疫苗反应能力的担忧。

相似文献

4
Pemphigus.天疱疮。
Nat Rev Dis Primers. 2017 May 11;3:17026. doi: 10.1038/nrdp.2017.26.

本文引用的文献

1
Impact of COVID-19 on autoimmune blistering diseases.COVID-19 对自身免疫性水疱病的影响。
Clin Dermatol. 2021 May-Jun;39(3):359-368. doi: 10.1016/j.clindermatol.2021.01.007. Epub 2021 Feb 2.

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验