Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom; National Institute of Health Research Biomedical Research Centre at Moorfields Eye Hospital NHS Foundation Trust and UCL Institute of Ophthalmology, London, United Kingdom; Singapore National Eye Centre and Singapore Eye Research Institute, Singapore.
King's College London, Mucosal and Salivary Biology, Dental Institute, London, United Kingdom; St. John's Institute of Dermatology, Guy's and St. Thomas's NHS Foundation Trust, London, United Kingdom.
Ophthalmology. 2018 Apr;125(4):496-504. doi: 10.1016/j.ophtha.2017.10.004. Epub 2017 Dec 6.
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.
Prospective, cross-sectional study.
Patients meeting the clinical criteria of ocular MMP, including those with positive and negative DIF findings.
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.
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.
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.
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.
本研究旨在探讨《黏膜性类天疱疮国际共识指南》（以下简称指南）的有效性。该指南建议，对于临床特征相似但直接免疫荧光（DIF）阴性的活检患者，应排除黏膜性类天疱疮（MMP）的诊断。如果存在眼部受累的 MMP 患者被误诊或延迟诊断，会导致不适当的局部治疗，而不是标准的 MMP 以外的其他病因所致的瘢痕性结膜炎的系统免疫调节治疗，从而导致 MMP 患者出现不可逆转的临床恶化。
前瞻性、横断面研究。
符合眼部 MMP 临床标准的患者，包括 DIF 阳性和阴性患者。
使用病例报告表收集人口统计学资料、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结果。除食管（以及部分患者的喉部）外，还检查了所有可能受 MMP 影响的解剖部位。记录 DIF 结果。
DIF 阳性和阴性患者在人口统计学、受累部位和疾病严重程度（通过结膜瘢痕程度（Tauber 分期）、中央角膜疾病（血管化、瘢痕、溃疡和结膜化）、结膜或眼睑手术史以及筛查时对全身免疫治疗的需求来确定）方面的差异。
共纳入 73 例眼部 MMP 患者，其中 73 例（27.4%）患者为单纯眼部疾病。DIF 阳性和阴性患者之间在人口统计学或临床方面没有显著差异。这一发现包括疾病严重程度的差异，唯一显著的差异是 DIF 阴性患者的中央角膜疾病更严重。不同部位的无症状疾病很常见。
这些发现不支持将 DIF 阴性、符合眼部 MMP 临床标准的患者归类为不同的疾病。为了临床管理目的，该类别患者应被视为 DIF 阴性 MMP，有炎症眼睛的患者应接受全身性免疫调节治疗。无症状的眼部、口腔和鼻咽部 MMP 频繁发现具有临床意义，意味着在无症状患者中应常规筛查这些部位。
