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自身免疫性大疱性疾病中与IgA/IgG沉积模式相关的人口统计学特征和疾病严重程度:一项基于登记数据库的队列研究

Demographic characteristics and disease severity associated with IgA/IgG deposition patterns in autoimmune bullous diseases: a cohort study based on a registry database.

作者信息

Li Jishu, Feng Xun, Wang Mi, Liu Hongjie, Yang Mei, Pang Jiyun, Zou Min, Xiao Yue, Zhang Xiwen, Hu Hongxiang, Zhou Yuxi, Alqusseireen Yazan Moufaq, Yan Wei, Zhou Xingli, Li Wei

机构信息

Department of Dermatology and Venereology and Rare Diseases Center, West China Hospital, Sichuan University, Chengdu, China.

Institutes for Systems Genetics, Frontiers Science Center for Disease-related Molecular Network, West China Hospital, Sichuan University, Chengdu, China.

出版信息

Front Immunol. 2025 May 26;16:1565073. doi: 10.3389/fimmu.2025.1565073. eCollection 2025.

DOI:10.3389/fimmu.2025.1565073
PMID:40491920
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12146370/
Abstract

BACKGROUND

Direct immunofluorescence (DIF) microscopy is the gold standard for diagnosing autoimmune bullous diseases (AIBDs), but the clinical significance of IgA and IgG co-deposition was unclear.

OBJECTIVE

Investigate the demographic differences and disease severity among different IgG/IgA deposition patterns in DIF.

METHODS

We conducted a retrospective cohort study based on a registry database that analyzed demographic data, involvement sites, and immunofluorescence patterns of patients with DIF biopsy. Patients were categorized into intercellular (group A) and basement membrane zone (group B) deposition patterns. Logistic regression models assessed associations between deposition status and demographic characteristics. Disease severity and prognosis were analyzed retrospectively through subgroup analyses.

RESULTS

In group A, female gender (OR = 1.665, = 0.011) and stronger IgG deposition (OR = 3.881, < 0.001) were associated with IgA and IgG co-deposition. In group B, female gender (OR = 1.382, = 0.002), stronger IgG deposition (OR = 2.673, < 0.001), and mucosa tissue (OR = 3.052, < 0.001) were associated with IgA and IgG co-deposition. IgA and IgG co-deposition in group A was associated with higher Pemphigus Disease Area Index scores ( = 0.036), while in group B, it correlated with mucosal involvement ( = 0.007). No differences in the proportion of disease severity scores improvement after 6 months of standard treatment were found in both groups.

CONCLUSIONS

Female gender, stronger IgG deposition, and mucosa tissue are key factors affecting IgA and IgG co-deposition in AIBD patients. For clinical correlation, patients with IgA and IgG co-deposition in pemphigus exhibit more severe disease severity compared with those with IgG deposition only, while patients with co-deposition in pemphigoid are more prone to mucosal involvement.

摘要

背景

直接免疫荧光(DIF)显微镜检查是诊断自身免疫性大疱性疾病(AIBDs)的金标准,但IgA和IgG共沉积的临床意义尚不清楚。

目的

研究DIF中不同IgG/IgA沉积模式之间的人口统计学差异和疾病严重程度。

方法

我们基于一个登记数据库进行了一项回顾性队列研究,该数据库分析了DIF活检患者的人口统计学数据、受累部位和免疫荧光模式。患者被分为细胞间(A组)和基底膜区(B组)沉积模式。逻辑回归模型评估沉积状态与人口统计学特征之间的关联。通过亚组分析对疾病严重程度和预后进行回顾性分析。

结果

在A组中,女性(OR = 1.665,P = 0.011)和更强的IgG沉积(OR = 3.881,P < 0.001)与IgA和IgG共沉积相关。在B组中,女性(OR = 1.382,P = 0.002)、更强的IgG沉积(OR = 2.673,P < 0.001)和黏膜组织(OR = 3.052,P < 0.001)与IgA和IgG共沉积相关。A组中IgA和IgG共沉积与更高的天疱疮疾病面积指数评分相关(P = 关于医学术语的准确性和专业性,天疱疮疾病面积指数评分相关的“ = 0.036”表述有误,应为具体数据,这里保留原文错误表述形式,实际翻译时应根据准确数据翻译),而在B组中,它与黏膜受累相关(P = 关于医学术语的准确性和专业性,这里的“ = 0.007”表述有误,应为具体数据,这里保留原文错误表述形式,实际翻译时应根据准确数据翻译)。两组在标准治疗6个月后疾病严重程度评分改善比例方面均未发现差异。

结论

女性、更强的IgG沉积和黏膜组织是影响AIBD患者IgA和IgG共沉积的关键因素。就临床相关性而言,与仅IgG沉积的患者相比,天疱疮中IgA和IgG共沉积的患者疾病严重程度更高,而类天疱疮中共沉积的患者更容易发生黏膜受累。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/75fa/12146370/c3c2d0768a48/fimmu-16-1565073-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/75fa/12146370/f55b4c7ac736/fimmu-16-1565073-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/75fa/12146370/c3c2d0768a48/fimmu-16-1565073-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/75fa/12146370/f55b4c7ac736/fimmu-16-1565073-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/75fa/12146370/c3c2d0768a48/fimmu-16-1565073-g002.jpg

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IgA autoantibody may be the foremost pathogenic in three cases of linear IgA/IgG bullous dermatosis.
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