A number of studies have evaluated QOL in AIBD by using generic tools such as the Medical Outcome Study 36\item Short Form (SF\36),174, 175, 176 Activities of Daily Living (ADLs),177 12\item General Health Questionnaire (GHQ\12),178, 179, 180 Hospital Anxiety and Depression scale (HADS),181, 182 Clinical Depression Questionnaire (CDQ),176, 183 Beck Depression Inventory (BDI),184, 185 and The Work Productivity and Activity Impairment QuestionnaireCSpecific Health Problem (WPAIQ\SHP).186, 187 The literature also mentions several dermatology\specific instruments that evaluate QOL in AIBD, including the Dermatology Life Quality Index (DLQI), Dermatology Quality of Life Scales, Dermatology Specific Quality of Life Instrument, Itchy QOL and Skindex\29.188, 189, 190, 191, 192, 193, 194, 195 The DLQI is the first validated dermatology\specific QOL instrument.188, 196 Currently, the SF\36 and the DLQI are the most often reported measures for evaluating QOL in AIBD. The Oral Health Impact Profile (OHIP)197 is the most common generic instrument used in the field of oral medicine.198 The OHIP was originally developed with 49 questions across seven domains; a shortened version was subsequently derived with 14 questions, referred to as OHIP\14.199 This was developed to provide a comprehensive measure of self\reported dysfunction, discomfort and disability resulting from oral conditions. involvement of the mucous membranes, such as the oral, ocular, nasal, nasopharyngeal, anogenital, laryngeal and oesophageal mucosa. MMP patients may present with mono\ or multisite involvement. Patients autoantibodies have been shown to be predominantly directed against BP180 (also called BPAG2, type XVII collagen), BP230, laminin 332 and type VII collagen, components of junctional adhesion complexes promoting epithelial stromal attachment in stratified epithelia. Numerous disease assessment scores are available, including the Mucous Membrane Pemphigoid Disease Area M344 Index (MMPDAI), the Autoimmune Bullous Skin M344 disorder Intensity Score (ABSIS), the Cicatrising Conjunctivitis Assessment Tool and the Oral Disease Severity Score (ODSS). Individual\reported end result measurements (PROMs), including DLQI, ABQOL and TABQOL, can be used for assessment of quality of life to evaluate the effectiveness of therapeutic interventions and monitor disease course. Introduction Mucous membrane pemphigoid (MMP) comprises a group of autoantibody\mediated subepidermal bullous diseases, characterized by a chronic course with predominant involvement of mucous membranes and a tendency to scarring.1, 2 Disease severity varies, ranging from mild/moderate disease with mild Rabbit polyclonal to EVI5L gingival inflammation, to severe disease affecting multiple mucosal surfaces. In 2002, a group of experts published a consensus\based guideline for the diagnosis and management of MMP.3 Advances regarding diagnosis and therapeutic options have led to the need for an update of the guideline, an initiative promoted by the European Academy of Dermatology and Venereology (EADV) Task Force for autoimmune bullous diseases (AIBD). The guideline is based on a systematic search and review of the available literature, in combination with a structured consensus process, resulting in a level S3 guideline. The full guideline document with methodology, search strategy, scientific questions and evidence furniture is usually available on the EADV website. It is the fourth guideline initiated by this EADV Task Force, following the guidelines for bullous pemphigoid (BP),4 pemphigus5 and dermatitis herpetiformis.6 Methods Guideline committee The guideline committee was established during the EADV Task Force meeting in Geneva, Switzerland, on 16 September 2017. The committee consisted of members of the EADV taskforce for AIBD, as well as selected specialists familiar with MMP, including dermatologists, ophthalmologists, oral medicine specialists, an otorhinolaryngologist and a pathologist. Several national patient businesses, including the German Pemphigus und Pemphigoid Selbsthilfegruppe e.V., the Dutch Netwerk voor Blaarziekten, the British PEM Friends and the Association Pemphigus Pemphigo?de France, as well as the International Pemphigus and Pemphigoid Foundation (IPPF), have reviewed the guideline. Finally, the European Dermatology Forum (EDF) has approved the final version. Conflict of interest forms were collected during the guideline process and approved by the guideline committee. Aim, scope and targeted target audience of the guideline During the guideline kick\off meeting in Groningen, The Netherlands, on 21 March M344 2018, the scope and methodology of the guideline and the targeted target audience were defined. The aim was to develop a multidisciplinary S3 European guideline, based on both evidence and expert opinion, and useful for all medical specialists who encounter patients with undiagnosed MMP, and treat and/or monitor patients with MMP. In this context, the overall aim is usually to provide specialists with a diagnostic and therapeutic algorithm. The professionals targeted are healthcare practitioners who may be involved with management of MMP patients in their daily practice. This includes general practitioners, dermatologists, ophthalmologists, otorhinolaryngologists, gynaecologists, urologists, gastroenterologists, dentists, oral medicine specialists and pathologists. Guideline methodology This guideline was developed in line with EDF standard operating procedures and in agreement with the quality criteria of the Appraisal of Guidelines Research & Evaluation II (AGREE II) instrument.7 Literature search During the guideline kick\off meeting, research queries were formulated. Searches were performed per formulated research question. The literature searches were performed in the MEDLINE and EMBASE (OvidSP) databases, published in European languages with no limitations in timeframe. To solution questions regarding management, we used the evidence explained in the Cochrane review of Kirtschig em et?al /em .8 and in the systematic review by Taylor em et?al /em .9 that updated the Cochrane search up to 2013. By using exactly the same keywords, we updated the search from 2013 until June 2019 in the.