Ormond M, McParland H, Thakrar P, Donaldson A N A, Andiappan M, Cook R J, Escudier M E, Higham J, Hullah E, McMillan R, Taylor J, Shirlaw P J, Challacombe S J, Setterfield J F
Department of Oral Medicine, St John's Institute of Dermatology, Guy's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, U.K.
Department of Oral Medicine, Birmingham Dental Hospital and School of Dentistry, Birmingham, U.K.
Br J Dermatol. 2020 Jul;183(1):78-85. doi: 10.1111/bjd.18566. Epub 2019 Nov 24.
Mucous membrane pemphigoid (MMP) is a rare autoimmune bullous disease predominantly affecting the oral mucosa. Optimal management relies upon thorough clinical assessment and documentation at each visit.
The primary aim of this study was to validate the Oral Disease Severity Score (ODSS) for the assessment of oral involvement in MMP. We also compared its inter- and intraobserver reliability with those of the oral parts of the Mucous Membrane Pemphigoid Disease Area Index (MMPDAI), Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Physician's Global Assessment (PGA).
Fifteen patients with mild-to-moderately severe oral MMP were scored for disease severity by 10 oral medicine clinicians from four U.K. centres using the ODSS, the oral sections of MMPDAI and ABSIS, and PGA. Two clinicians rescored all patients after 2 h.
In terms of reliability, the interobserver ODSS total score intraclass correlation coefficient (ICC) was 0·97, MMPDAI activity 0·59 and damage 0·15, ABSIS total 0·84, and PGA 0·72. The intraobserver ICCs (two observers) for ODSS total were 0·97 and 0·93; for MMPDAI activity 0·93 and 0·70 and damage 0·93 and 0·79; for ABSIS total 0·99 and 0·94; and for PGA 0·92 and 0·94. Convergent validity between ODSS and MMPDAI was good (correlation coefficient 0·88). The mean ± SD time for completion of ODSS was 93 ± 31 s, with MMPDAI 102 ± 24 s and ABSIS involvement 71 ± 18 s. The PGA took < 5 s.
This study has validated the ODSS for the assessment of oral MMP. It has shown superior interobserver agreement over MMPDAI, ABSIS and PGA, and superior intraobserver reliability to MMPDAI. It is quick and easy to perform. What's already known about this topic? There are no validated scoring methodologies for oral mucous membrane pemphigoid (MMP). Proposed disease activity scoring tools for MMP include the Mucous Membrane Disease Area Index (MMPDAI) and the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). The Oral Disease Severity Score (ODSS) has been validated for use in oral pemphigus vulgaris (PV). It has been shown to be reliable and sensitive in both lichen planus (LP) and MMP. What does this study add? The ODSS has been shown to be a thorough, sensitive and reproducible, yet quick scoring tool for the assessment of oral involvement in MMP. Its versatility for use in oral PV, MMP and LP is an added advantage over other scoring methodologies. What are the clinical implications of this work? We propose that the ODSS be used as a clinical scoring tool for monitoring activity in oral MMP in clinical practice as well as for use in multicentre studies.
黏膜类天疱疮（MMP）是一种罕见的自身免疫性大疱性疾病，主要累及口腔黏膜。最佳治疗方案依赖于每次就诊时的全面临床评估和记录。
本研究的主要目的是验证口腔疾病严重程度评分（ODSS）在评估MMP口腔受累情况中的作用。我们还将其观察者间和观察者内的可靠性与黏膜类天疱疮疾病面积指数（MMPDAI）、自身免疫性大疱性皮肤病严重程度评分（ABSIS）及医生整体评估（PGA）的口腔部分进行了比较。
来自英国四个中心的10名口腔医学临床医生使用ODSS、MMPDAI和ABSIS的口腔部分以及PGA，对15例轻至中度严重口腔MMP患者的疾病严重程度进行评分。两名临床医生在2小时后对所有患者重新评分。
在可靠性方面，观察者间ODSS总分的组内相关系数（ICC）为0.97，MMPDAI活动度为0.59，损害度为0.15，ABSIS总分为0.84，PGA为0.72。ODSS总分的观察者内ICC（两名观察者）分别为0.97和0.93；MMPDAI活动度分别为0.93和0.70，损害度分别为0.93和0.79；ABSIS总分为0.99和0.94；PGA为0.92和0.94。ODSS与MMPDAI之间的收敛效度良好（相关系数0.88）。完成ODSS的平均±标准差时间为93±31秒，MMPDAI为102±24秒，ABSIS受累情况为71±18秒。PGA用时<5秒。
本研究验证了ODSS在评估口腔MMP中的作用。它在观察者间一致性方面优于MMPDAI、ABSIS和PGA，在观察者内可靠性方面优于MMPDAI。它操作快速简便。关于该主题已知的信息有哪些？目前尚无用于口腔黏膜类天疱疮（MMP）的经过验证的评分方法。提议的MMP疾病活动度评分工具包括黏膜疾病面积指数（MMPDAI）和自身免疫性大疱性皮肤病严重程度评分（ABSIS）。口腔疾病严重程度评分（ODSS）已被验证可用于寻常型口腔天疱疮（PV）。它在扁平苔藓（LP）和MMP中均显示出可靠且敏感。本研究增加了什么内容？ODSS已被证明是一种全面、敏感且可重复，但快速的评分工具，用于评估MMP的口腔受累情况。它在口腔PV、MMP和LP中的通用性是相对于其他评分方法的一个额外优势。这项工作的临床意义是什么？我们建议将ODSS用作临床评分工具，用于在临床实践中监测口腔MMP的活动情况以及用于多中心研究。
