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皮肤补体沉积的存在可区分大疱性类天疱疮的免疫学和组织学特征——一项回顾性队列研究的见解

Presence of Cutaneous Complement Deposition Distinguishes between Immunological and Histological Features of Bullous Pemphigoid-Insights from a Retrospective Cohort Study.

作者信息

Ständer Sascha, Holtsche Maike M, Schmidt Enno, Hammers Christoph M, Zillikens Detlef, Ludwig Ralf J, Kridin Khalaf

机构信息

Department of Dermatology, University of Lübeck, 23562 Lübeck, Germany.

Lübeck Institute of Experimental Dermatology, University of Lübeck, 23562 Lübeck, Germany.

出版信息

J Clin Med. 2020 Dec 3;9(12):3928. doi: 10.3390/jcm9123928.

Abstract

The practical implications of complement deposition in direct immunofluorescence (DIF) microscopy and its influence on the disease phenotype are poorly understood. We aimed to investigate whether the presence of complement deposition in DIF microscopy gives rise to differences in the morphological, immunological, and histological characteristics of patients with BP (bullous pemphigoid). We performed a retrospective study encompassing patients with BP in a specialized tertiary referral center. Logistic regression model was utilized to identify variables independently associated with complement deposition. The study included 233 patients with BP, of whom 196 (84.1%) demonstrated linear C3 deposition along the dermal-epidermal junction (DEJ) in DIF analysis. BP patients with C3 deposition had higher mean (SD) levels (645.2 (1418.5) vs. 172.5 (243.9) U/mL; < 0.001) and seropositivity rate (86.3% vs.64.9%; = 0.002) of anti-BP180 NC16A and less prevalent neutrophilic infiltrate in lesional skin specimens (29.8% vs. 52.4%; = 0.041). C3 deposition was found positively associated with the detection of anti-BP180 NC16A autoantibodies (OR, 4.25; 95% CI, 1.38-13.05) and inversely associated with the presence of neutrophils in lesional skin (OR, 3.03; 95% CI, 1.09-8.33). To conclude, complement deposition influences the immunological and histological features of BP. These findings are in line with experimental data describing the pathogenic role of complement in BP.

摘要

直接免疫荧光(DIF)显微镜检查中补体沉积的实际意义及其对疾病表型的影响尚不清楚。我们旨在研究DIF显微镜检查中补体沉积的存在是否会导致大疱性类天疱疮(BP)患者在形态学、免疫学和组织学特征上的差异。我们在一家专业的三级转诊中心对BP患者进行了一项回顾性研究。采用逻辑回归模型来确定与补体沉积独立相关的变量。该研究纳入了233例BP患者,其中196例(84.1%)在DIF分析中显示沿真皮-表皮交界处(DEJ)有线性C3沉积。有C3沉积的BP患者抗BP180 NC16A的平均(标准差)水平更高(645.2(1418.5)对172.5(243.9)U/mL;P<0.001)、血清阳性率更高(86.3%对64.9%;P = 0.002),且皮损标本中嗜中性粒细胞浸润较少见(29.8%对52.4%;P = 0.041)。发现C3沉积与抗BP180 NC16A自身抗体的检测呈正相关(比值比,4.25;95%可信区间,1.38 - 13.05),与皮损中嗜中性粒细胞的存在呈负相关(比值比,3.03;95%可信区间,1.09 - 8.33)。总之,补体沉积影响BP的免疫学和组织学特征。这些发现与描述补体在BP中的致病作用的实验数据一致。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/453f/7761814/be549a3d0232/jcm-09-03928-g001.jpg

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