Autoantibody Profile Differentiates between Inflammatory and Noninflammatory Bullous Pemphigoid
Autoantibody Profile Differentiates between Inflammatory and Noninflammatory Bullous Pemphigoid
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DOI：
10.1016/j.jid.2016.06.622
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发表时间：
2016-11-01
影响因子：
6.5
通讯作者：
Shimizu, Hiroshi
中科院分区：
文献类型：
--
作者：
Izumi, Kentaro;Nishie, Wataru;Shimizu, Hiroshi
大疱性类天疱疮（Bullous Pemphigoid，BP）是一种主要的自身免疫性水疱性皮肤病，其中大多数自身抗体（autoAb）靶向半桥粒胶原XVII的跨膜细胞外非胶原16 A结构域（NC 16 A）。BP-autoAbs可能靶向胶原蛋白XVII的区域而不是NC 16 A结构域;然而，BP-autoAbs的表位与临床特征之间的相关性尚未完全阐明。为了解决BP-自身抗体的临床特征和特异性表位之间的相关性，我们评估了121例患者的BP-自身抗体的表位谱。共有87例患者表现出典型的炎症表型，红斑和autoAb靶向抗NC 16 A结构域，而14例患者表现出明显的非炎症表型，其中autoAb特异性靶向胶原XVII的中间部分，但不靶向NC 16 A。有趣的是，该组临床上显示与嗜酸性粒细胞的少量病变浸润相关的红斑显著减少。令人惊讶的是，14例病例中有7例（50.0%）接受了二肽基肽酶-IV抑制剂治疗糖尿病。二肽基肽酶-IV抑制剂用于3/76例（3.9%）具有靶向NC 16 A的自身抗体的典型BP病例;因此，二肽基肽酶-IV抑制剂被认为参与非典型非炎性BP的发展。这项研究表明，autoAb谱区分炎症和非炎症BP，而非炎症BP可能与二肽基肽酶-IV抑制剂。
Bullous pemphigoid (BP) is a major autoimmune blistering skin disorder, in which a majority of the autoantibodies (autoAbs) target the juxtamembranous extracellular noncollagenous 16A domain (NC16A) domain of hemidesmosomal collagen XVII. BP-autoAbs may target regions of collagen XVII other than the NC16A domain; however, correlations between epitopes of BP-autoAbs and clinical features have not been fully elucidated. To address correlations between the clinical features and specific epitopes of BP-autoAbs, we evaluated the epitope profiles of BP-autoAbs in 121 patients. A total of 87 patients showed a typical inflammatory phenotype with erythema and autoAbs targeting the anti-NC16A domain, whereas 14 patients showed a distinct noninflammatory phenotype, in which autoAbs specifically targeted the midportion of collagen XVII, but not NC16A. Interestingly, this group clinically showed significantly reduced erythema associated with scant lesional infiltration of eosinophils. Surprisingly, 7 of the 14 cases (50.0%) received dipeptidyl peptidase-IV inhibitors for the treatment of diabetes. Dipeptidyl peptidase-IV inhibitors were used in 3 of 76 (3.9%) typical cases of BP with autoAbs targeting NC16A; thus, dipeptidyl peptidase-IV inhibitors are thought to be involved in the development of atypical noninflammatory BP. This study shows that the autoAb profile differentiates between inflammatory and noninflammatory BP, and that noninflammatory BP may be associated with dipeptidyl peptidase-IV inhibitors.