Birmingham and Midland Eye Centre

BACKGROUND: Cataract is a leading cause of vision impairment in older adults, affecting almost half of those over age 75 years. Driving is a highly visual task and, as with other age groups, older adults rely on the personal automobile for travel. The purpose of this study was to examine the role of cataract in driving. METHODS: Older adults (aged 55-85 years) with cataract (n = 279) and those without cataract (n = 105) who were legally licensed to drive were recruited from eye clinics to participate in a driving habits interview to assess driving status, exposure, difficulty, and "space" (the distance of driving excursions from home base). Crash data over the prior 5 years were procured from state records. Visual functional tests documented the severity of vision impairment. RESULTS: Compared to those without cataract, older drivers with cataract were approximately two times more likely to report reductions in days driven and number of destinations per week, driving slower than the general traffic flow, and preferring someone else to drive. Those with cataract were five times more likely to have received advice about limiting their driving. Those with cataract were four times more likely to report difficulty with challenging driving situations, and those reporting driving difficulty were two times more likely to reduce their driving exposure. Drivers with cataract were 2.5 times more likely to have a history of at-fault crash involvement in the prior 5 years (adjusted for miles driven/week and days driven/week). These associations remained even after adjustments for the confounding effects of advanced age, impaired general health, mental status deficit, or depression. CONCLUSIONS: Older drivers with cataract experience a restriction in their driving mobility and a decrease in their safety on the road. These findings serve as a baseline for our ongoing study evaluating whether improvements in vision following cataract surgery expand driving mobility and improve driver safety.

BACKGROUND/AIMS: Uveitis is a major cause of visual morbidity in the working age group. The authors investigated the duration, degree, and causes of visual loss in uveitis patients with the aim of better defining the visual morbidity and identifying potential risk factors. METHODS: A retrospective, non-interventional, observational survey of 315 consecutive patients attending a tertiary referral uveitis service. RESULTS: The mean duration of follow up was 36.7 months. Reduced vision (< or =6/18) was found in 220/315 (69.95%) of the patients with a subset of 120 patients having vision < or =6/60. Unilateral visual loss occurred in 109 (49.54%), while 111 (50.45%) had bilateral loss. The mean duration of visual loss was 21 months. Of the 148 patients with pan-uveitis, 125 (84.45%) had reduced vision, with 66 (53%) having vision < or =6/60. Main causes of visual loss were cystoid macular oedema (CMO) (59/220, 26.8%), cataract (39/220, 17.7%), and combination of CMO and cataract (44/220, 20%). The following were predictive of a poorer visual prognosis: pan-uveitis (p = 0.0005), bilateral inflammation (p = 0.0005), increasing duration of reduced vision (p = 0.0005), an Indian or Pakistani ethnic background (p = 0.004), and increasing patient age (p = 0.02). CONCLUSION: Prolonged visual loss occurred in two thirds of uveitis patients, with 70 (22%) patients meeting the criteria for legal blindness at some point in their follow up. Older patients with bilateral inflammation and an increasing duration of reduced vision are at the greatest risk of severe visual loss (< or =6/60). CMO and cataract were responsible for visual loss in 64.5% of patients.

Behçet's disease (BD), also known as the Silk Road disease, is a blinding inflammatory disorder of young adults found predominantly between the Mediterranean basin and the Orient, and is strongly associated with the major histocompatibility complex (MHC) antigen HLA-B51. In this article we review the history of Behçet's disease since its first description by Hippocrates, the development of the trading routes collectively known as the Silk Road and the effect of population movement on the distribution of HLA-B51. The global distribution of this antigen among healthy control populations bears a striking similarity both to the ancient trading routes and the distribution of Behçet's disease, suggesting a genetic risk that migrated in parallel with population movement between the Mediterranean and Asia. However, certain indigenous Amerindian peoples have a high prevalence of HLA-B51 but no reported cases of BD. Furthermore, a clear genealogical relationship exists between eastern, but not central, Siberian populations with the Amerindians. Since a high level of recombination within the MHC is known to have occurred in these eastern populations before their migration into Beringia, we suggest that disruption of genetic loci in linkage disequilibria with HLA-B51 may be one reason for the absence of disease in these high HLA-B51-bearing populations. However, a contributory influence of environmental factors is not excluded by this data, and the wide variation that exists in relative risk of HLA-B51 even within Europe would support other non-genetic risk factors on the Silk Road which may be absent, or non-contributory to disease, in the Americas.

PURPOSE: To compare hysteresis, a novel measure of ocular rigidity (viscoelasticity) in normal and keratoconic eyes. METHODS: The study consisted of 207 normal and 93 keratoconic eyes. Eyes were diagnosed as keratoconic based on clinical examination and corneal topography. The hysteresis was measured by the Ocular Response Analyzer (ORA; Reichert Ophthalmic Instruments, Buffalo, NY). The data were recorded by Generation 3 software for the ORA. Central corneal thickness (CCT) was measured with a handheld ultrasonic pachymeter in the midpupillary axis. RESULTS: The mean hysteresis was 10.7 +/- 2.0 (SD) mm Hg (range, 6.1-17.6) in normal eyes compared with 9.6 +/- 2.2 mm Hg (range, 4.7-16.7) in keratoconic eyes. The difference was statistically significant (P < 0.0001, unpaired t-test). Mean CCT in the normal and keratoconic eyes was 545.0 +/- 36.4 microm (range, 471-650) and 491.8 +/- 54.7 microm (range, 341-611), respectively; the difference was significant (P < 0.0001, unpaired t-test). CONCLUSIONS: Hysteresis was significantly higher in normal than in keratoconic eyes. It may be a useful measurement in addition to CCT, when assessing ocular rigidity, and may be of particular importance when trying to correct intraocular measurements for increased or decreased ocular rigidity. Long-term studies of change in hysteresis may provide information on the progression of keratoconus.

Antimicrobial resistance (AMR) is a serious threat to global public health, with approximately 5 million deaths associated with bacterial AMR in 2019. Tackling AMR requires a multifaceted and cohesive approach that ranges from increased understanding of mechanisms and drivers at the individual and population levels, AMR surveillance, antimicrobial stewardship, improved infection prevention and control measures, and strengthened global policies and funding to development of novel antimicrobial therapeutic strategies. In this rapidly advancing field, this Review provides a concise update on AMR, encompassing epidemiology, evolution, underlying mechanisms (primarily those related to last-line or newer generation of antibiotics), infection prevention and control measures, access to antibiotics, antimicrobial stewardship, AMR surveillance, and emerging non-antibiotic therapeutic approaches. The Review also discusses the potential roles of artificial intelligence in addressing AMR, including antimicrobial susceptibility testing, AMR surveillance, antimicrobial stewardship, diagnosis, and antimicrobial drug discovery and development. This Review highlights the urgent need for addressing the global effects of AMR and for rapid advancement of relevant technology in this dynamic field.

Discussion of the pathogenesis of syringomyelia involves considering the origin of the fluid and also the forces which cause that fluid to break down the structure of the cord. When cerebrospinal fluid (CSF) appears to be the destructive element, it commonly enters through a patent central canal running from the fourth ventricle to the inside of the syrinx. In both clinical and experimental situations pressure differences may be measured which suck on the hindbrain, particularly the cerebellar tonsils, producing deformities. These pressure differences may also suck fluid into the syrinx. In other cases, even when a communication does not appear to be patent, the hindbrain abnormalities are usually present and suck effect may usually be demonstrated and its correction be accompanied by clinical improvement. Other sources of fluid within a syrinx include liquefaction of haematomata after traumatic paraplegia and transudation of fluid from intrinsic spinal tumours. Once fluid is present within a cord cavity it may pulsate upwards and downwards in response to fluid movements in the subarachnoid space, the most energetic of which result from venous influences. Such movement, ‘slosh’, may cause the cavities to extend at either end giving rise to upward and downward extension from a post-traumatic cord cyst and sometimes to syringobulbia. Cord ischaemia, venous congestion and transport of fluid along perivascular spaces may all play a part in the maintainance of cord cavities or the progression of the clinical disabilities.

Antibodies to ubiquitin have been used to search for evidence of abnormal protein degradation in amyotrophic lateral sclerosis--motor neuron disease (ALS). Anterior horn cell ubiquitin-immunoreactive (IR) inclusions were present in all of 31 ALS cases but in none of 23 neurologically normal and in only 1 of 22 neurologically abnormal controls. These inclusions, which were present in familial and sporadic ALS cases, and in cases with dementia, took the form of dense rounded or irregular ubiquitin-IR cytoplasmic inclusions (dense bodies), or loosely arranged bundles ('skeins') of filamentous-appearing material. The presence of ubiquitin-IR inclusions corresponded to the pattern of selective neuronal vulnerability in ALS, although inclusions in pyramidal neurons of the motor cortex were infrequent and were noted in only a minority of cases. Ubiquitin-IR inclusions were more prevalent than Bunina bodies. The latter were present in 67% of ALS cases but were seldom labelled by antibodies to ubiquitin. Intraneuronal inclusions resembling Lewy bodies were present in 23% of ALS cases and were often identified by antibodies to ubiquitin. We conclude that the presence of ubiquitin-IR inclusions in lower motor neurons represents a characteristic pathological feature of ALS in its various clinical forms. Ubiquitin-IR inclusions in ALS differ from ubiquitinated inclusions in other neuronal degenerations in that they are not readily identified by antibodies to cytoskeletal proteins. They may represent accumulations of altered or abnormal neuronal proteins resistant to degradation via the ubiquitin proteolytic pathway.

We present a novel indentation method for characterizing the viscoelastic properties of alginate and agarose hydrogel based constructs, which are often used as a model system of soft biological tissues. A sensitive long working distance microscope was used for measuring the time-dependent deformation of the thin circular hydrogel membranes under a constant load. The deformation of the constructs was measured laterally. The elastic modulus as a function of time can be determined by a large deformation theory based on Mooney-Rivlin elasticity. A viscoelastic theory, Zener model, was applied to correlate the time-dependent deformation of the constructs with various gel concentrations, and the creep parameters can therefore be quantitatively estimated. The value of Young's modulus was shown to increase in proportion with gel concentration. This finding is consistent with other publications. Our results also showed the great capability of using the technique to measure gels with incorporated corneal stromal cells. This study demonstrates a novel and convenient technique to measure mechanical properties of hydrogel in a non-destructive, online and real-time fashion. Thus this novel technique can become a valuable tool for soft tissue engineering.

RAT RETINA 613 cataract than the retinal lesion itself. This point is being more fully investigated.

Artificial intelligence (AI) is expected to improve healthcare outcomes by facilitating early diagnosis, reducing the medical administrative burden, aiding drug development, personalising medical and oncological management, monitoring healthcare parameters on an individual basis, and allowing clinicians to spend more time with their patients. In the post-pandemic world where there is a drive for efficient delivery of healthcare and manage long waiting times for patients to access care, AI has an important role in supporting clinicians and healthcare systems to streamline the care pathways and provide timely and high-quality care for the patients. Despite AI technologies being used in healthcare for some decades, and all the theoretical potential of AI, the uptake in healthcare has been uneven and slower than anticipated and there remain a number of barriers, both overt and covert, which have limited its incorporation. This literature review highlighted barriers in six key areas: ethical, technological, liability and regulatory, workforce, social, and patient safety barriers. Defining and understanding the barriers preventing the acceptance and implementation of AI in the setting of healthcare will enable clinical staff and healthcare leaders to overcome the identified hurdles and incorporate AI technologies for the benefit of patients and clinical staff.

PURPOSE: To extensively characterize the complex network of cytokines present in uveitis aqueous humor (AqH), and the relationships between cytokines and the cellular infiltrate. METHODS: AqH from noninflammatory control subjects and patients with idiopathic, Fuchs' heterochromic cyclitis (FHC), and herpes-viral or Behçet's uveitis were analyzed for IL-1beta, -2, -4, -5, -7, -8, -10, -12, -13, -15, TNFalpha, IFNgamma, CCL2 (MCP-1), CCL5 (RANTES), CCL11 (Eotaxin), TGFbeta2, and CXCL12 (SDF-1), using multiplex bead immunoassays. The cellular infiltrate was also determined for each sample. RESULTS: Idiopathic uveitis AqH, compared with noninflammatory controls, was characterized by high levels of IL-6, IL-8, CCL2 and IFNgamma, the levels of which correlated with each other. For IL-6 and IL-8 these levels were proportional to the number of neutrophils present. By contrast, the levels of both TGFbeta2 and CXCL12 decreased in idiopathic uveitis AqH with increasing inflammation. Cluster analysis showed a degree of segregation between noninflammatory and idiopathic uveitis AqH. Further examination using random forest analysis yielded a complete distinction between these two groups. The minimum cytokines required for this classification were IL-6, IL-8, CCL2, IL-13, TNFalpha, and IL-2. CONCLUSIONS: Application of multiplex bead immunoassays has allowed us to identify distinct patterns of cytokines that relate to both clinical disease and the cellular infiltrates present. Bioinformatics analysis allowed identification of cytokines that differentiate idiopathic uveitis from noninflammatory control AqH and are likely to be important for the pathogenesis of uveitis.

The use of drains in the treatment of syringomyelia has a simple and immediate appeal and has been practiced widely since the report of Abbe and Coley over 100 years ago. Good short-term results have been claimed in the past, but long-term outcome is largely unknown. An experience in Birmingham, England is reviewed in which 73 patients who had had some form of syrinx drainage procedure performed were subsequently followed up. In these cases, a total of 56 syringopleural and 14 syringosubarachnoid shunts had been inserted. Ten years after the operations, only 53.5% and 50% of the patients, respectively, continued to remain clinically stable. A 15.7% complication rate was recorded, including fatal hemorrhage, infection, and displacement of the drain from the pleural and syrinx cavities. At second operation or necropsy, at least 5% of shunts were discovered to be blocked. The effect of other drainage procedures that do not use artificial tubing, such as syringotomy and terminal ventriculostomy, was analyzed but found not to offer any substantial benefit. These results indicate that drainage procedures are not an effective solution to remedying the progressive, destructive nature of syringomyelia. It is suggested that, rather than attempting to drain the syrinx cavity, disabling the filling mechanism of the syrinx is more appropriate. Most forms of syringomyelia have a blockage at the level of the foramen magnum or in the subarachnoid space of the spine. Surgical measures that aim to reconstruct the continuity of the subarachnoid space at the site of the block are strongly recommended. Lowering the overall pressure of the cerebrospinal fluid is advocated when reestablishment of the pathways proves impossible. Syrinx drainage as an adjuvant to more physiological surgery may have a place in the treatment of syringomyelia. If two procedures are done at the same time, however, it is difficult to ascribe with certainty a success or failure, and it is suggested that the drainage procedure be reserved for a later attempt if the elective first operation fails.

The clinical details of 18 cases of brainstem encephalitis are presented. Correlation of the clinical course with radiological and pathological findings confirms that this is not a variant of polyradiculitis or the Guillain-Barré syndrome, but is a distinct clinical entity which carries a benign prognosis.

CSF pressure recordings have been taken from the lumbar region and the cisterna magna of 16 patients during coughing in the sitting position. Isolated coughs of low amplitude have been studied. The lumbar pressure waves occur sooner and lower. Thus there is a phase during which the lumbar pressure exceeds the cisternal, followed by one in which the cisternal exceeds the lumbar. These phenomena may be conveniently displayed on a differential trace. The phase during which the cisternal pressure exceeds the lumbar may be protracted. It is suggested that Froin's syndrome, central subarachnoid pouches and syringobulbia may be associated with upward pressure waves. Cough headache, the filling stages of communicating syringomyelia and tonsillar herniation may be associated with valve-like blockage at the foramen magnum which produces craniospinal pressure dissociation by interfering with downward or rebound pulsation. Decompensation of hydrocephalus after birth may be related to pulsation in association with crying; also after removal of a meningocele sac decompensation may be related to the effects of similar pulsation modified by changes in capacitance following operation. The cord destruction of syringomyelia, and the mechanisms which fill spinal subarachnoid cysts may be related to pressure waves directed both upwards and downwards. The merits and limitations of cough impulse as a clinical test for spinal blockage are discussed, and the suggestion is made that after further evaluation they may provide a more sensitive indication of spinal blockage than Queckenstedt's test.

Seven patients with rheumatoid disease were given the iron-chelating drug desferrioxamine (DFX) to evaluate its possible anti-inflammatory effects. Two of these patients, who also received the anti-emetic prochlorperazine, lost consciousness for 48-72 h and then fully recovered. Electroencephalography showed abnormalities of the type associated with metabolic disturbance. One of these patients showed pyramidal features and subsequently developed an optic neuropathy and pigmentary retinopathy. Analysis of his cerebrospinal fluid showed a decrease in loosely-bound (catalytic) iron and increase in loosely-bound (catalytic) copper, total iron and products of lipid peroxidation, with values approaching normal as the symptoms resolved. Subsequent in vivo/vitro studies clearly demonstrated that the neurological effects were due to a synergistic action of desferrioxamine and prochlorperazine, probably resulting in exceptional fluxes of intra/extra cellular iron/copper disturbing noradrenergic and serotonergic systems. Two other patients who did not receive prochlorperazine, developed retinal problems which later improved, one after only 15 g of desferrioxamine. Our observations suggest a new model for metabolic encephalopathy studies and provide insight into the mechanisms of pigmentary retinopathy.

Visual acuity (VA) is paramount to outcome measures in ophthalmic studies. The Early Treatment Diabetic Retinopathy Study (ETDRS) chart is internationally recognized as ‘gold standarnumerical imputation for low vision stad’ for VA measurement in clinical trials and population surveys [ICO 1984 + 2002 reports]. It utilizes the logarithm of the minimum angle of resolution (LogMAR); a continuous measure of VA with a linear scale. In contrast, Snellen fraction uses a geometric progression, which is inherently skewed, however, remains commonly reported in clinical practice. When performing statistical analysis, non-conversion of Snellen fraction to LogMAR results in erroneous results and misrepresentative statistical analyses (Holladay 1997; Holladay 2004; Yu & Afifi 2009). Therefore, it becomes necessary to convert Snellen fraction to LogMAR accurately when using retrospective data (Holladay 1997; Holladay 2004). Online conversion tables and calculators are available albeit impractical; they are tedious for large databases and do not account for additional or missed letters. For additional or missing letters, the most common methodology employs ± 0.02 LogMAR per missing/gained letter as per Ferris et al. (1982). Furthermore, in patients with poor vision, the resolving visual acuities’ ordinal scale of ‘counting fingers’ (CF), ‘hand movement’ (HM), ‘light perception’ (LP) and ‘no light perception’ (NLP) are employed. These values commonly are quantified and converted to a numerical form permitting statistical analysis (Grover et al. 1999; Holladay 2004; Schulze-Bonsel et al. 2006; Bach 2007; Lange et al. 2009; Day et al. 2015). Multiple authors have recommended or used various conversions which are summarized in Table 1. NLP would intuitively equal 0.0 decimal VA, which is non-quantifiable in LogMAR units. Whilst CF and HM are measurable with acceptable test-retest repeatability, LP and NLP are not (Schulze-Bonsel et al. 2006). Schulze and Bach (co-author for Schulze-Bonsel et al. (2006)) propose that three lines on a LogMAR chart are generally clinically significant (Schulze-Bonsel et al. 2006), consequently assigning LP and NLP numerical imputations of 0.3 and 0.6 LogMAR units below HM, respectively (Bach 2007). The National Ophthalmology Database (NOD) has published many papers for numerous cases in the UK, consistently using the reference values found in Table 1 for their publications. These referenced values have been utilized in other large case-series publications (Chu et al. 2016). 0.014 (decVA) 1.85 LogMAR 0.005 (decVA) 2.30 LogMAR 433 On: 06/06/20 246 On 06/06/20 National ophthalmology database. Values consistently used in every publication from this group to allow comparison Reason for reference values, not stated. 112 On 06/06/20 144 On 06/06/20 498 On 06/06/20 Microsoft Excel® (Microsoft Corporation, Redmond, WA) is a spreadsheet program that is widely used for data collection and basic analysis. A recent publication by colleagues outlined a method using Excel to convert Snellen fraction to LogMAR (Tiew et al. 2020). This method, however, is limited in that it does not allow direct entry of the Snellen fraction into one cell. For example, ‘6/9-2’ would need to be entered across three different cells (‘6’, ‘9’, ‘-2’). Moreover, this method does not allow for conversion of non-numerical values such as CF, HM, LP and NLP. We demonstrate a user-friendly and efficient Excel sheet® method, enabling conversion of Snellen fraction and non-numerical VA values to LogMAR. This formula converts one cell of Excel to another cell and can be copy pasted and dragged down a column to convert large data of Snellen fraction rapidly and effectively including low VA, to LogMAR. Our Excel formula has several advantages including: A downloadable Excel Sheet (Appendix S1) will enable customization of the formula in terms of reference LogMAR for CF, HM, LP and NLP. It allows re-labelling, for example LP to PL, as well as editing the starting reference cell (currently A2). By default, the formula utilizes the United Kingdom NOD values (Table 1; Day et al. 2015). Figure 1 outlines more detailed instructions and illustrates use. We exercise caution in performing statistical analysis particularly with VA < HM. As no author has reliably and reproducibly converted LP and NLP, these are numerical imputations to aid quantification in database analysis. Consequently, we recommend avoiding parametric analysis and utilizing non-parametric analyses to interpret values as ordinal data if converting to LogMAR for statistical analysis. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

The last years have seen a major interest in designing and deploying trust management and public key infrastructures. Yet, it is still far from clear how one can pass from the organization and system requirements to the actual credentials and attribution of permissions in the PKI infrastructure.\nOur goal in this paper is filling this gap. We propose a formal framework for modeling and analyzing security and trust requirements, that extends the Tropos methodology for early requirements modeling. The key intuition that underlies our work is the identification of distinct roles for actors that manipulate resources, accomplish goals or execute tasks, and actors that own or permit usage of resources or goals. The paper also presents a simple case study and a PKI/trust management implementation.

Abstract This guideline has been initiated by the task force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology, including physicians from all relevant disciplines and patient organizations. It is a S3 consensus‐based guideline that systematically reviewed the literature on mucous membrane pemphigoid (MMP) in the MEDLINE and EMBASE databases until June 2019, with no limitations on language. While the first part of this guideline addressed methodology, as well as epidemiology, terminology, aetiology, clinical presentation and outcome measures in MMP, the second part presents the diagnostics and management of MMP. MMP should be suspected in cases with predominant mucosal lesions. Direct immunofluorescence microscopy to detect tissue‐bound IgG, IgA and/or complement C3, combined with serological testing for circulating autoantibodies are recommended. In most patients, serum autoantibodies are present only in low levels and in variable proportions, depending on the clinical sites involved. Circulating autoantibodies are determined by indirect IF assays using tissue substrates, or ELISA using different recombinant forms of the target antigens or immunoblotting using different substrates. The major target antigen in MMP is type XVII collagen (BP180), although in 10–25% of patients laminin 332 is recognized. In 25–30% of MMP patients with anti‐laminin 332 reactivity, malignancies have been associated. As first‐line treatment of mild/moderate MMP, dapsone, methotrexate or tetracyclines and/or topical corticosteroids are recommended. For severe MMP, dapsone and oral or intravenous cyclophosphamide and/or oral corticosteroids are recommended as first‐line regimens. Additional recommendations are given, tailored to treatment of single‐site MMP such as oral, ocular, laryngeal, oesophageal and genital MMP, as well as the diagnosis of ocular MMP. Treatment recommendations are limited by the complete lack of high‐quality randomized controlled trials.

Aims-To estimate the prevalence of Usher syndrome in the city of Birmingham, and to establish a database of patients who have been classified into diVerent clinical subtypes essential for future gene mutation analysis. Methods-Symptomatic cases of Usher syndrome (US) resident in the city of Birmingham in June 1994 were ascertained through multiple sources. Ophthalmic and audiological reassessment together with examination of medical records and patient questionnaires allowed classification of three subtypes, US 1, US 2, and US 3. In addition, family pedigrees were examined and blood was taken from index patients for DNA extraction.

This guideline on mucous membrane pemphigoid (MMP) has been elaborated by the Task Force for Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology (EADV) with a contribution of physicians from all relevant disciplines and patient organizations. It is a S3 consensus-based guideline encompassing a systematic review of the literature until June 2019 in the MEDLINE and EMBASE databases. This first part covers methodology, the clinical definition of MMP, epidemiology, MMP subtypes, immunopathological characteristics, disease assessment and outcome scores. MMP describes a group of autoimmune skin and mucous membrane blistering diseases, characterized by a chronic course and by predominant 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. Various disease assessment scores are available, including the Mucous Membrane Pemphigoid Disease Area Index (MMPDAI), the Autoimmune Bullous Skin disorder Intensity Score (ABSIS), the 'Cicatrising Conjunctivitis Assessment Tool' and the Oral Disease Severity Score (ODSS). Patient-reported outcome 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.