pemphigoid

类天疱疮
  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    关于亚洲自身免疫性大疱性皮肤病(AIBDs)住院患者住院和死亡率相关因素的数据有限。本研究旨在阐明影响亚洲AIBDs患者住院率和死亡率的危险因素。使用国际疾病分类第10次修订代码对在Siriraj医院接受治疗的AIBDs患者进行了17年的回顾性分析。比较住院和门诊患者的特点,并确定了死亡率和相关因素。该研究包括360名AIBD患者(180名住院患者,180名门诊病人)。住院患者明显比门诊患者年轻。已确定的住院危险因素是恶性肿瘤(比值比[OR]2.83,95%置信区间[CI]1.13-8.04;p=0.034),中度至重度疾病(OR2.52,95%CI1.49-4.34;p<0.001),全身使用皮质类固醇≥15mg/天(OR2.27,95%CI1.21-4.41;p=0.013)和口服环磷酰胺治疗(OR9.88,95%CI3.82-33.7;p<0.001).Kaplan-Meier分析显示死亡率为26%,36%和39%的住院天疱疮患者在1年,3年和5年,分别。对于类天疱疮患者,相应的比率为28%,38%和47%。感染,尤其是肺炎,是两种情况下死亡的主要原因。这项研究证实,亚洲种族和医疗保健差异可能与AIBDs患者的不良后果有关。亚洲患者的天疱疮死亡率明显高于白种人。持续监测导致住院和死亡的因素对于改善治疗结果至关重要。
    Limited data exist on the factors associated with hospitalization and mortality in Asian inpatients with autoimmune bullous dermatoses (AIBDs). This study aimed to elucidate the risk factors affecting hospitalization and mortality rates in Asian patients with AIBDs. A retrospective analysis of patients with AIBDs treated at Siriraj Hospital during a 17-year period was performed using the International Classification of Diseases 10th revision codes. The characteristics of inpatients and outpatients were compared, and mortality rates and associated factors were identified. The study included 360 AIBD patients (180 inpatients, 180 outpatients). Inpatients were significantly younger than outpatients. The identified risk factors for hospitalization were malignancy (odds ratio [OR] 2.83, 95% confidence interval [CI] 1.13-8.04; p = 0.034), moderate to severe disease (OR 2.52, 95% CI 1.49-4.34; p < 0.001), systemic corticosteroid use ≥15 mg/day (OR 2.27, 95% CI 1.21-4.41; p = 0.013) and oral cyclophosphamide treatment (OR 9.88, 95% CI 3.82-33.7; p < 0.001). Kaplan-Meier analysis revealed mortality rates of 26%, 36% and 39% for inpatients with pemphigus at 1, 3 and 5 years, respectively. For inpatients with pemphigoid, the corresponding rates were 28%, 38% and 47%. Infections, particularly pneumonia, were the predominant cause of death in both conditions. This study confirmed that both Asian ethnicity and healthcare disparities may be correlated with adverse outcomes in patients with AIBDs. Pemphigus mortality rates were substantially greater in Asian patients than in Caucasian patients. Continuous monitoring of factors contributing to hospitalization and mortality is imperative to improve treatment outcomes.
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  • 文章类型: Journal Article
    患有自身免疫性大疱性疾病的患者感染风险增加,来自潜在的皮肤病和免疫抑制治疗。关于皮肤科患者,尤其是自身免疫性大疱性疾病的成年人的疫苗信念和行为的信息有限。为了了解疫苗决策,识别疫苗接种的感知风险和益处,并讨论美国自身免疫性大疱性疾病患者的个人经历。利用半结构化访谈进行了定性研究,并对NVivo进行了分析。在编码数据中识别了模式,并记录了每个主要主题的代表性报价。面试于2022年2月15日至2022年9月15日进行。20例诊断为大疱性类天疱疮的患者,粘膜类天疱疮,寻常型天疱疮,或天疱疮叶状接受了采访。在20名参与者中,14人(70%)为女性,带有平均值(SD,范围)年龄64.8(13.2,34-83)岁。从访谈的定性分析中得出的关键主题包括患者对感染易感性增加的担忧,疫苗接种后皮肤疾病的潜在恶化,以及免疫抑制药物对疫苗体液反应的影响。缺乏预约可用性,难以获得疫苗,和费用通常被认为是疫苗接种的障碍。这些发现为皮肤科医生提供了宝贵的知识,可以提供针对患者问题的咨询,并改善皮肤科环境中疫苗接种的沟通。
    Patients with autoimmune bullous diseases are at an increased risk of infection, both from the underlying skin disease and from immunosuppressive treatments. Limited information is available on vaccine beliefs and behaviors in dermatology patients and adults with autoimmune bullous diseases in particular. To understand vaccine decision making, identify perceived risks and benefits of vaccinations, and discuss individual experiences in patients with autoimmune bullous diseases in the United States. A qualitative study was performed utilizing semi-structured interviews, and analysis was conducted on NVivo. Patterns were identified in the coded data, and representative quotations were recorded for each major theme. Interviews were conducted between February 15, 2022 and September 15, 2022. Twenty patients with a diagnosis of bullous pemphigoid, mucous membrane pemphigoid, pemphigus vulgaris, or pemphigus foliaceous were interviewed. Of the 20 participants, 14 (70%) were female, with a mean (SD, range) age of 64.8 (13.2, 34-83) years. Key themes that emerged from qualitative analysis of the interviews included patient concerns regarding their increased susceptibility to infection, potential exacerbation of skin disease following vaccination, and the effect of immunosuppressive medications on humoral response to vaccines. Lack of appointment availability, difficulty accessing vaccines, and cost were commonly identified barriers to vaccination. These findings provide valuable knowledge for dermatologists in regard to providing counseling specific to patient concerns and to improve communication surrounding vaccination in the dermatology setting.
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  • 文章类型: Journal Article
    天疱疮和类天疱疮是全身性大疱性自身免疫性疾病,影响皮肤和/或粘膜,具有危及生命的性质,尤其是寻常型天疱疮.皮肤科医生和口腔科医生发表的论文优先代表了他们对皮肤粘膜疾病的关注。
    本研究的目的是比较皮肤科医生和口腔科医生在Scopus数据库中发表的天疱疮和类天疱疮的科学计量特征。
    皮肤科医生和口腔科医生发表了9276篇和760篇论文,分别。在2007-2022年期间,皮肤科医生的年度出版物数量从218稳定增加到526;而在此期间,口腔科医生的数量从18增加到51。对于引用最多的前200篇论文,皮肤科医生的天疱疮出版物的引用总数为42,766,h指数为148;而口腔科医生的出版物的引用总数为14,689,h指数为63。值得注意的是,天疱疮的最初迹象经常出现在口腔粘膜,表现为红斑,水泡,以及口腔溃疡,牙龈炎,扁平苔藓样天疱疮.
    本研究首次报道了皮肤科医生和口腔科医生发表的天疱疮的科学计量特征。皮肤科医生的出版物的规模和引用大大超过了口腔科医生的出版物,建议口腔科医生可以从皮肤科医生那里学习,并在天疱疮研究方面进行更多合作。
    UNASSIGNED: Pemphigus 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.
    UNASSIGNED: The objective of this study was to compare the scientometric characteristics of pemphigus and pemphigoid publications by dermatologists and stomatologists in the Scopus database.
    UNASSIGNED: There 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.
    UNASSIGNED: This 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.
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  • 文章类型: Journal Article
    回顾性分析dupilumab治疗大疱性类天疱疮的疗效和安全性。
    回顾性收集2020年10月至2022年10月在我科接受dupilumab治疗的大疱性类天疱疮患者的病历,分析其治疗效果及实验室指标变化。
    回顾了总共11例接受dupilumab治疗的大疱性类天疱疮患者的记录。在治疗的2周内,11例患者中有10例(90.9%)完全或基本控制了疾病。患者的BPDAI评分在治疗后2周时从基线113(62,181)降至37(6,130)(p=.001),在治疗后12周时降至4(0,37)(p<.001)。在接受dupilumab治疗的11例患者中,瘙痒缓解时间为0-3天(0.5,7)天,2周后瘙痒明显缓解(t=15.925,p<.001)。DLQI评分从治疗前的(25.5±2.5)分下降,至2周时的(11.8±4.4)(t=10.764,p<.001)和12周时的(2.1±1.9)(t=30.038,p<.001)。患者的嗜酸性粒细胞计数较高,高血清IgE水平,低血清总蛋白水平,凝血功能异常。治疗后上述指标逐渐恢复正常。治疗过程中无不良反应发生。
    Dupilumab能有效控制大疱性类天疱疮的病情,有效缓解瘙痒症状,相对安全。
    To retrospectively analyze the efficacy and safety of dupilumab in the treatment of bullous pemphigoid.
    From October 2020 to October 2022, the medical records of patients with bullous pemphigoid who were treated with dupilumab in our department were collected retrospectively to analyze the therapeutic effect and changes in laboratory indexes.
    The records of a total of 11 patients with bullous pemphigoid who were treated with dupilumab was reviewed. Within 2 weeks of the treatment, 10 (90.9%) of the 11 patients had complete or substantial control of the disease. The BPDAI scores of the patients decreased from baseline 113 (62, 181) to 37 (6, 130) at 2 weeks (p = .001) and 4 (0, 37) at 12 weeks after treatment (p < .001). In the 11 patients treated with dupilumab, the relief time of pruritus was 0-3 days (0.5, 7) days, and the pruritus was significantly alleviated after 2 weeks (t = 15.925, p < .001). The DLQI score decreased from (25.5 ± 2.5) before treatment, to (11.8 ± 4.4) at 2 weeks (t = 10.764, p < .001) and (2.1 ± 1.9) at 12 weeks (t = 30.038, p < .001). The patients had high eosinophil counts, high serum IgE levels, low serum total protein levels, and abnormal blood coagulation function. The aforementioned indicators gradually returned to normal after treatment. No adverse reactions occurred during the treatment.
    Dupilumab can effectively control the condition of bullous pemphigoid, efficiently relieve pruritus symptoms, and is relatively safe.
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  • 文章类型: Journal Article
    自身免疫性起泡(AIBD)疾病影响粘膜,皮肤,和附属物。迄今为止,尚未广泛研究阑尾受累。然而,它们很重要,因为在疾病发作期间通常会遇到指甲变化。
    确定各种免疫球性疾病中指甲变化的患病率和模式,并研究指甲变化与疾病持续时间和严重程度之间的关系。
    在皮肤科进行了一项横断面研究,venerology,以及孟买三级护理中心的麻风病。
    进行了一项横断面研究,其中包括总共74例免疫性疾病,并确定了指甲变化的患病率。使用ANOVA(方差分析)和非配对t检验分析指甲变化的平均数与疾病持续时间和严重程度之间的关联。
    社会研究统计软件包(SPSS)软件用于统计评价。
    我们发现指甲变化的患病率为91%。相对于AIBD中粘膜受累的严重程度,指甲变化的平均数存在显着差异(P值<0.05)。关于寻常型天疱疮(PV)的严重程度,指甲变化的平均数没有显着差异,天疱疮(PF),表皮下起泡疾病,以及AIBD的持续时间。
    指甲在AIBD中经常受到影响。指甲变化的数量与粘膜疾病的严重程度有关,但与持续时间无关。
    UNASSIGNED: Autoimmune blistering (AIBD) disorders affect mucosae, skin, and appendages. Appendageal involvement has not been studied extensively to date. However, they are important as nail changes are commonly encountered during a flare-up of the disease.
    UNASSIGNED: To determine the prevalence and patterns of nail changes in various Immunobullous disorders and to study the relationship between the nail changes and the disease duration and severity.
    UNASSIGNED: A cross-sectional study was conducted at the Department of Dermatology, venerology, and leprosy at a tertiary care center in Mumbai.
    UNASSIGNED: A cross-sectional study including a total of 74 cases of Immunobullous diseases was conducted and the prevalence of nail changes was determined. The association between the mean number of nail changes and the disease duration and severity was analyzed using ANOVA (Analysis of variance) and unpaired t-test.
    UNASSIGNED: The Statistical Package for Social Studies (SPSS) software was used for statistical evaluation.
    UNASSIGNED: We found that the prevalence of nail changes was 91%. There was a significant difference in the mean number of nail changes with respect to the severity grades of mucosal involvement in AIBD (P value < 0.05). There was no significant difference in the mean number of nail changes with respect to the severity grades of Pemphigus vulgaris (PV), Pemphigus foliaceous (PF), subepidermal blistering diseases, and the duration of AIBD.
    UNASSIGNED: Nails are frequently affected in AIBD. The number of nail changes is related to the severity of mucosal disease but not to duration.
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