Validation of database search strategies for the epidemiological study of pemphigus and pemphigoid.
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ABSTRACT:
SUBMITTER: Hsu D
PROVIDER: S-EPMC5216167 | biostudies-literature | 2016 Mar
REPOSITORIES: biostudies-literature
Publications
Validation of database search strategies for the epidemiological study of pemphigus and pemphigoid.
Hsu D D Brieva J J Nardone B B Silverberg J I JI
The British journal of dermatology 20151117 3
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Project description:ObjectivesThe validity of bullous pemphigoid and pemphigus vulgaris recording in routinely collected healthcare data in the UK is unknown. We assessed the positive predictive value (PPV) for bullous pemphigoid and pemphigus vulgaris primary care Read codes in the Clinical Practice Research Datalink (CPRD) using linked inpatient data (Hospital Episode Statistics (HES)) as the diagnostic benchmark.SettingAdult participants with bullous pemphigoid or pemphigus vulgaris registered with HES-linked general practices in England between January 1998 and December 2017. Code-based algorithms were used to identify patients from the CPRD and extract their benchmark blistering disease diagnosis from HES.Primary outcome measureThe PPVs of Read codes for bullous pemphigoid and pemphigus vulgaris.ResultsOf 2468 incident cases of bullous pemphigoid and 431 of pemphigus vulgaris, 797 (32.3%) and 85 (19.7%) patients, respectively, had a hospitalisation record for a blistering disease. The PPV for bullous pemphigoid Read codes was 93.2% (95% CI 91.3% to 94.8%). Of the bullous pemphigoid cases, 3.0% had an HES diagnosis of pemphigus vulgaris and 3.8% of another blistering disease. The PPV for pemphigus vulgaris Read codes was 58.5% (95% CI 48.0% to 68.9%). Of the pemphigus vulgaris cases, 24.7% had an HES diagnosis of bullous pemphigoid and 16.5% of another blistering disease.ConclusionsThe CPRD can be used to study bullous pemphigoid, but recording of pemphigus vulgaris needs to improve in primary care.
| S-EPMC7365488 | biostudies-literature
Project description:Autoimmune bullous dermatoses (AIBDs) are a group of rare chronic inflammatory skin diseases, which clinically manifest as blisters and erosions of the skin and/or mucosa. Immunologically, AIBDs are characterized and caused by autoantibodies targeting adhesion molecules in the skin and mucosa. According to the histological location of the blistering, AIBDs are classified into the following two main subtypes: pemphigus (intraepidermal blistering) and pemphigoid (subepidermal blistering). Most AIBDs were potentially life-threatening diseases before the advent of immunosuppressive drugs, especially systemic steroid therapies, which suppress pathogenic immunological activity. Although there have been recent advancements in the understanding of the pathogenesis of AIBDs, glucocorticosteroids and/or adjuvant immunosuppressive drugs are still needed to control disease activity. However, the long-term use of systemic immunosuppression is associated with major adverse events, including death. Based on the growing understanding of AIBD pathogenesis, novel treatment targets have emerged, some of which are currently being evaluated in clinical trials. Within this article, we review the current clinical trials involving pemphigus and pemphigoid and discuss the rationale that lead to these trials. Overall, we aim to foster insights into translational research in AIBDs to improve patient care.
| S-EPMC6509547 | biostudies-literature
Project description:Pemphigus and bullous pemphigoid are autoantibody-mediated blistering skin diseases. In pemphigus, keratinocytes in epidermis and mucous membranes lose cell-cell adhesion, and in pemphigoid, the basal keratinocytes lose adhesion to the basement membrane. Pemphigus lesions are mediated directly by the autoantibodies, whereas the autoantibodies in pemphigoid fix complement and mediate inflammation. In both diseases, the autoantigens have been cloned and characterized; pemphigus antigens are desmogleins (cell adhesion molecules in desmosomes), and pemphigoid antigens are found in hemidesmosomes (which mediate adhesion to the basement membrane). This knowledge has enabled diagnostic testing for these diseases by enzyme-linked immunosorbent assays and dissection of various pathophysiological mechanisms, including direct inhibition of cell adhesion, antibody-induced internalization of antigen, and cell signaling. Understanding these mechanisms of disease has led to rational targeted therapeutic strategies.
| S-EPMC5560122 | biostudies-other
Project description:BackgroundPrevious case-control studies have suggested that environmental factors including exposure to pesticides and organic materials, diet and medications have an important role in the pathogenesis of pemphigus vulgaris. These studies lacked geographical population controls and had less than three controls per case.ObjectiveTo identify environmental and occupational risk factors associated with the development of pemphigus vulgaris (PV) and bullous pemphigoid (BP).MethodCases were patients with PV (n = 25) and BP (n = 29) recruited from 2009 to 2017. Controls for PV (n = 72) and BP (n = 84) were recruited from the general population via electoral commission matching, matched for age, sex, residential location, and ethnicity. Data about demographics, environmental exposures and occupational exposures, was collected using a structured questionnaire. Conditional logistic regression analysis was undertaken using SPSS software to identify significant variables.ResultsSignificant factors associated with PV included the daily consumption of leeks (odds ratio (OR) 3.6; p = 0.025), mustard oil (OR = 4.4; p = 0.049), tomatoes (OR = 4.735; p = 0.032), multivitamins (OR 3.6; p = 0.009), alcohol (0.039), and calcium supplements (OR = 44, p < 0.001). Other associated factors included the number of lifetime sunburns (p = 0.019), high levels of mental stress (p < 0.001), and the use of lime household cleaning products (p < 0.001), Significant factors associated with BP included the daily consumption of green or herbal tea (OR = 3.7; p = 0.004), fish oil (OR = 5.7; p < 0.001), calcium supplements (OR = 6.1; p < 0.001), multivitamins (OR = 2.6; p = 0.043), and glucosamine (OR = 3.0; p = 0.046). The use of lime household cleaning products (p < 0.001) and high levels of mental stress (p = 0.007) were also associated with BP.ConclusionDietary factors containing thiol groups such as leeks, tomatoes, and mustard oil may be potential triggers for PV. High levels of mental stress, the use of supplementary medications such as calcium and multivitamins, and chemical cleaning products containing lime may be associated with an increased risk of developing both PV and BP. Lifestyle changes should be part of routine management for these patients.
| S-EPMC11537152 | biostudies-literature
Project description:Background/purposePemphigus and pemphigoid are systemic bullous autoimmune diseases affecting skin and/or mucosal membranes with the life-threatening nature, especially pemphigus vulgaris. The papers published by dermatologists and stomatologists preferentially represent their concerns of a mucocutaneous disease.Materials and methodsThe objective of this study was to compare the scientometric characteristics of pemphigus and pemphigoid publications by dermatologists and stomatologists in the Scopus database.ResultsThere are 9276 and 760 papers published by dermatologists and stomatologists, respectively. The annual number of the publications by dermatologists stably raised from 218 to 526 during 2007-2022; while the number by stomatologists raised with a small amount from 18 to 51 during this period. For the most-cited top-200 papers, the total citation count is 42,766 and the h index is 148 for pemphigus publications by dermatologists; whereas the count is 14,689 and h index is 63 for publications by stomatologists. Notably, first signs of pemphigus often appear in oral mucosa, manifesting as erythema, blisters, as well as mouth ulcer, gingivitis, lichen planus-like pemphigus.ConclusionThis study firstly reports the scientometric characteristics of pemphigus publications by dermatologists and stomatologists. The scale and citations of dermatologists' publications greatly outweigh stomatologists' ones, suggesting stomatologists can learn from and more cooperate with dermatologists regarding pemphigus research.
| S-EPMC10547994 | biostudies-literature
Project description:Pemphigus and pemphigoid are paradigms for understanding the mechanisms of antibody-mediated autoimmune disease in humans. In pemphigus, IgG4-predominant autoantibodies cause intraepidermal blistering by direct interference with desmoglein interactions and subsequent disruption of desmosomes and signaling pathways. In pemphigoid, IgG1, IgG4, and IgE autoantibodies against basement membrane zone antigens directly interfere with hemidesmosomal adhesion, activating complement and Fc receptor‒mediated effector pathways. Unraveling disease mechanisms in pemphigus and pemphigoid has identified numerous opportunities for clinical trials, which hold promise to identify safer and more effective therapies for these potentially life-threatening diseases.
| S-EPMC8860856 | biostudies-literature
Project description:Pemphigus and pemphigoid diseases are organ-specific autoimmune diseases of the skin and/or mucous membranes, which are caused by autoantibodies targeting structural proteins of the skin. In other autoimmune diseases, a high prevalence of primary antibody deficiencies was noted. Conversely, a high prevalence of autoimmune diseases is reported in patients with primary antibody deficiencies. With the exception of one study, pointing toward a decrease of IgG in pemphigus patients, with a relative enrichment of IgG4, serum immunoglobulin (Ig) concentrations had not been studied in pemphigus and pemphigoid. Hence, we here aimed to investigate serum concentrations of IgM, IgA, IgG, and IgG1-4 in pemphigus and pemphigoid patients, as well as in healthy controls. Serum Ig concentrations were determined by ELISA in 105 healthy controls, 100 pemphigus vulgaris (PV), 100 pemphigus foliaceus, 99 bullous pemphigoid (BP), and 55 linear IgA bullous dermatosis (LAD) patients. In healthy controls, age had a significant impact on Ig serum concentrations: In controls at ages of 69 years or older, IgM and IgG were decreased, while all other Ig, except IgA and IgG4, were increased. When stratified by sex, lower IgM concentrations were observed in males. When corrected for age and/or sex, and compared to controls, an increase in serum IgA was noted in LAD. In almost all patient cohorts, an increase in IgG1 and IgG4 was observed, while a decrease in IgG2 or IgG3 was seen in BP or PV patients. This points toward a possible association of BP with IgG2 deficiency and warrants evaluation of IgG2 in BP patients prior to immunosuppressive therapy.
| S-EPMC7493804 | biostudies-literature
Project description:Autoimmune bullous disorders are a heterogeneous spectrum of skin disorders characterized by the production of autoantibodies against adhesion molecules of the skin. The 2 major groups of diseases are “pemphigus diseases” and “autoimmune bullous diseases of the pemphigoid type.” Pemphigus diseases are a group of autoimmune blistering diseases of the skin and mucous membranes characterized by intraepithelial cleft and acantholysis. The main subtypes of pemphigus include pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. Diagnosis is based on clinical manifestations and confirmed with histological, immunofluorescence, and serological testing. Recently multivariant enzyme-linked immunosorbent assay systems have been developed as practical screening tools for patients with suspected autoimmune bullous dermatoses. The current first-line treatment of pemphigus is based on systemic corticosteroids that are often combined with immunosuppressive adjuvants, such as azathioprine, mycophenolate mofetil, and the anti-CD20 monoclonal antibody rituximab, usually at initiation of treatment. Rituximab efficacy is higher when it is administered early in the course of the disease. Therefore, it should be used as first-line treatment to improve efficacy and reduce cumulative doses of corticosteroids and their side effects. Treatment of bullous pemphigoid is based on disease extension. Localized and mild forms can be treated with superpotent topical corticosteroids or with nonimmunosuppressive agents. In patients with generalized disease or whose disease is resistant to the treatments described above, systemic corticosteroids are preferred and effective. Adjuvant immunosuppressants are often combined with steroids for their steroid-sparing effect.
| S-EPMC7319750 | biostudies-literature
Project description:The 5th Scientific Conference of the International Pemphigus and Pemphigoid Foundation (IPPF), "Pemphigus and Pemphigoid: A New Era of Clinical and Translational Science" was held in Orlando, Florida, on May 15-16, 2018. Scientific sessions covered recent, ongoing, and future clinical trials in pemphigus and bullous pemphigoid, disease activity and quality of life instruments, and the IPPF Natural History Study. Furthermore, the meeting provided an opportunity to hear firsthand from patients, investigators, and industry about their experience enrolling for clinical trials.
| S-EPMC6236000 | biostudies-literature
Project description:Pemphigus encompasses a heterogeneous group of autoimmune blistering diseases, which affect both mucous membranes and the skin. The disease usually runs a chronic-relapsing course, with a potentially devastating impact on the patients' quality of life. Pemphigus pathogenesis is related to IgG autoantibodies targeting various adhesion molecules in the epidermis, including desmoglein (Dsg) 1 and 3, major components of desmosomes. The pathogenic relevance of such autoantibodies has been largely demonstrated experimentally. IgG autoantibody binding to Dsg results in loss of epidermal keratinocyte adhesion, a phenomenon referred to as acantholysis. This in turn causes intra-epidermal blistering and the clinical appearance of flaccid blisters and erosions at involved sites. Since the advent of glucocorticoids, the overall prognosis of pemphigus has largely improved. However, mortality persists elevated, since long-term use of high dose corticosteroids and adjuvant steroid-sparing immunosuppressants portend a high risk of serious adverse events, especially infections. Recently, rituximab, a chimeric anti CD20 monoclonal antibody which induces B-cell depletion, has been shown to improve patients' survival, as early rituximab use results in higher disease remission rates, long term clinical response and faster prednisone tapering compared to conventional immunosuppressive therapies, leading to its approval as a first line therapy in pemphigus. Other anti B-cell therapies targeting B-cell receptor or downstream molecules are currently tried in clinical studies. More intriguingly, a preliminary study in a preclinical mouse model of pemphigus has shown promise regarding future therapeutic application of Chimeric Autoantibody Receptor T-cells engineered using Dsg domains to selectively target autoreactive B-cells. Conversely, previous studies from our group have demonstrated that B-cell depletion in pemphigus resulted in secondary impairment of T-cell function; this may account for the observed long-term remission following B-cell recovery in rituximab treated patients. Likewise, our data support the critical role of Dsg-specific T-cell clones in orchestrating the inflammatory response and B-cell activation in pemphigus. Monitoring autoreactive T-cells in patients may indeed provide further information on the role of these cells, and would be the starting point for designating therapies aimed at restoring the lost immune tolerance against Dsg. The present review focuses on current advances, unmet challenges and future perspectives of pemphigus management.
| S-EPMC6603181 | biostudies-literature