Medical Student, University of Illinois at Chicago College of Medicine, Chicago, Illinois, USA.
Department of Dermatology, Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.
Ocul Immunol Inflamm. 2024 Dec;32(10):2521-2534. doi: 10.1080/09273948.2024.2397715. Epub 2024 Sep 6.
Ocular predominant mucous membrane pemphigoid (oMMP) is a severe subtype of MMP that can lead to scarring and blindness. While conjunctival biopsy for direct immunofluorescence (DIF) is considered the gold standard for diagnosis, limited sensitivity results in a false-negative rate upwards of 40%. Likewise, it remains unclear to what extent a negative biopsy, whether false-negative or true-negative, results in a different prognosis, with patients previously termed "pseudopemphigoid" demonstrating comparable disease progression. Serologic testing allows for a less invasive means to demonstrate circulating autoantibodies against known autoantigens in pemphigoid diseases. Patients with MMP, particularly oMMP, however, typically demonstrate low titers of circulating autoantibodies, limiting the diagnostic utility of these tests. The autoantigen integrin β4 has been previously reported to be a specific marker of pure ocular MMP, while in the majority of patients with oMMP, the identified target antigens are BP180 (type XVII collagen) and laminin 332. Recent studies have, however, demonstrated inconsistent reactivity and specificity for integrin β4 as an ocular-specific marker in MMP. Herein, we review the role of serologic testing in the diagnosis and prognosis of oMMP, as well as the current understanding of autoantigens in oMMP.: BMZ - basement membrane zone, DIF - direct immunofluorescence, IIF - indirect immunofluorescence, MMP - mucous membrane pemphigoid, oMMP - ocular predominant mucous membrane pemphigoid.
眼型黏膜性类天疱疮（oMMP）是一种严重的 MMP 亚型，可导致瘢痕和失明。虽然结膜直接免疫荧光（DIF）活检被认为是诊断的金标准，但敏感性有限，导致假阴性率高达 40%以上。同样，阴性活检（无论是假阴性还是真阴性）在多大程度上导致不同的预后尚不清楚，以前被称为“假性类天疱疮”的患者表现出相似的疾病进展。血清学检测可通过较少的侵袭性手段来证明在类天疱疮疾病中循环自身抗体针对已知自身抗原的存在。然而，患有 MMP，特别是 oMMP 的患者，通常表现出循环自身抗体的低滴度，限制了这些检测的诊断效用。整合素β4 自身抗原以前被报道为纯眼型 MMP 的特异性标志物，而在大多数 oMMP 患者中，鉴定的靶抗原是 BP180（XVII 型胶原）和层粘连蛋白 332。然而，最近的研究表明，整合素β4 作为 MMP 中眼特异性标志物的反应性和特异性不一致。在此，我们回顾了血清学检测在 oMMP 的诊断和预后中的作用，以及对 oMMP 中自身抗原的当前认识：BMZ - 基底膜区，DIF - 直接免疫荧光，IIF - 间接免疫荧光，MMP - 黏膜性类天疱疮，oMMP - 眼型黏膜性类天疱疮。
