Purpose

This study explored the validity of the First International Consensus on Mucous Membrane Pemphigoid (MMP) guidance, which recommends that clinically indistinguishable patients, who have direct immunofluorescence (DIF)-negative biopsies, be excluded from a diagnosis of MMP. Misdiagnosis, or delayed diagnosis, of MMP with ocular involvement leads to the inappropriate use of topical therapy, the standard of care for causes of cicatrising conjunctivitis other than MMP, rather than systemic immunomodulatory therapy, resulting in irreversible clinical deterioration in patients with MMP.

Design

Prospective, cross-sectional study.

Participants

Patients meeting the clinical criteria of ocular MMP, including those with positive and negative DIF findings.

Methods

A case report form was used to collect the demographic details, the clinical history, and the results of a detailed clinical assessment by ophthalmologists, otolaryngologists, dermatologists, and oral medicine specialists. All anatomic sites potentially affected by MMP were examined apart from the esophagus (and larynx in a subset). The DIF results were recorded.

Main Outcome Measures

Differences between DIF-positive and -negative patients in demography, sites of involvement, and disease severity as determined by the degree of conjunctival scarring (using Tauber staging), central corneal disease (vascularization, scarring, ulceration, and conjunctivalization), history of conjunctival or lid surgery, and requirement for systemic immunotherapy at the time of screening.

Results

A total of 73 patients with ocular MMP were recruited, of whom 20 of 73 (27.4%) had ocular-only disease. There was no significant demographic or clinical difference between patients with positive and negative DIF results. This finding included differences in disease severity for which the only significant difference was that of more severe central corneal disease in DIF-negative patients. Asymptomatic disease at different sites was frequent.

Conclusions

These findings do not support the classification of DIF-negative patients, meeting the clinical criteria for ocular MMP, as having a different disease. This category of patients should be accepted as having DIF-negative MMP, for clinical management purposes, with patients having inflamed eyes being treated with systemic immunomodulatory therapy. The frequent finding of asymptomatic ocular, oral, and nasopharyngeal MMP is clinically significant and implies that these sites should be routinely screened in asymptomatic patients.

中文翻译：

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眼膜粘液膜天疱疮

目的

这项研究探讨了关于粘膜膜天疱疮（MMP）的第一份国际共识的有效性，该指南建议将具有直接免疫荧光（DIF）阴性活检的临床上无法区分的患者排除在MMP的诊断之外。眼部累及MMP的误诊或延误诊断会导致局部治疗的不适当地使用，这是除MMP以外的治疗结膜炎性结膜炎的标准护理方法，而非全身性免疫调节疗法，导致MMP患者的临床不可逆转恶化。

设计

前瞻性，横断面研究。

参加者

符合眼部MMP临床标准的患者，包括DIF阳性和阴性的患者。

方法

病例报告表用于收集人口统计学细节，临床病史以及眼科医生，耳鼻喉科医生，皮肤科医生和口腔医学专家进行的详细临床评估的结果。除了食管（和子集中的喉部）外，还检查了所有可能受MMP影响的解剖部位。记录DIF结果。

主要观察指标

DIF阳性和阴性患者的人口统计学，受累部位和疾病严重程度之间的差异，取决于结膜瘢痕形成程度（采用陶伯分期法），中央角膜疾病（血管形成，瘢痕形成，溃疡和结膜形成），结膜病史或眼睑手术，以及在筛查时需要全身免疫治疗。

结果

总共招募了73例眼部MMP患者，其中73例中只有20例（27.4％）患有仅眼病。DIF结果阳性和阴性的患者在人口统计学或临床上无显着差异。这一发现包括疾病严重程度的差异，唯一的显着差异是DIF阴性患者中更严重的中央角膜疾病的差异。在不同部位无症状性疾病很常见。

结论

这些发现不支持DIF阴性患者的分类，因为其患有不同的疾病，因此符合眼部MMP的临床标准。出于临床管理的目的，应将这类患者的DIF阴性MMP接受，并采用全身免疫调节疗法治疗眼睛发炎的患者。无症状的眼，口腔和鼻咽MMP的频繁发现在临床上具有重要意义，并暗示对无症状患者应常规筛查这些部位。