大疱性类天疱疮患者疱液中趋化因子受体5和可诱导共刺激分子的检测

目的：检测C?X?C趋化因子受体5（CXCR5）和可诱导共刺激分子（ICOS）在大疱性类天疱疮（BP）患者疱液中的水平,探讨其在BP发病机制中的意义。方法收集15例BP患者疱液为实验组,15例Ⅱ度烧伤患者疱液为对照组,采用酶联免疫吸附测定（ELISA）方法分别检测CXCR5及ICOS在两组疱液中的水平。结果实验组及对照组疱液中CXCR5水平分别为（219±145.31）、（147±23.83）ng/L,差异有统计学意义（t=4.577,P<0.05）,但疱液中ICOS水平差异无统计学意义[（30.18±14.86）比（21.43±5.32）ng/L,t=1.628,P>0.05]。结论 CXCR5的表达可能与BP发病有一定关系,而ICOS与BP发病的关系尚需进一步研究。     

常见口腔黏膜疾病患者行种植修复相关问题的研究进展

随着修复技术的发展及人们对生活质量要求的提高,伴有缺牙的口腔黏膜病患者行种植修复的需求越来越高.受口腔黏膜病困扰的缺牙患者行种植修复是否可行,效果如何目前并无定论,亦无针对口腔黏膜病患者行种植修复的相关治疗指南.本文就常见的口腔黏膜病(如口腔扁平苔藓、自身免疫性大疱性疾病和舍格伦综合征等)患者行种植修复的相关研究进展进...     

Therapy of autoimmune mouth bullous disease: 2020 review

Most diseases of oral mucosa are either autoimmune in nature or are the results of immunologically mediated events. The diseases with autoimmune pathogenesis are namely pemphigus and pemphigoid; the oral involvement is frequent or regularly observed in these diseases. The treatments with traditional drugs or biologic agents or combinations of these molecules are employed in clinical practice. New therapeutic targets aim to provide new treatment strategies that may go beyond nonspecific immunosuppression.     

金匮肾气丸与糖皮质激素配伍治疗大疱性类天疱疮临床疗效观察

目的 观察金匮肾气丸联合糖皮质激素治疗大疱性类天疱疮的临床疗效、激素用量,探讨中西医结合方法治疗类天疱疮的可行性、安全性.方法 选择经临床及病理诊断的大疱性类天疱疮30例,随机分为2组,对照组给予泼尼松0.75 mg/(kg·d),研究组在此基础上加服金匮肾气丸1丸/次,2次/d.采用《大疱病临床研究观察表》记录患者每天新发水疱数、全身总水疱数和激素剂量等.结果 两组患者每天新发水疱数、全身总水疱数均减少,研究组较对照组水疱数减少更为明显(P＜0.01),但激素剂量和不良反应均小于对照组(P＜0.01).结论 金匮肾气丸与糖皮质激素配伍治疗类天疱疮具有疗效高、安全性高、不良反应小的特点,是值得临床推广的中西医结合治疗手段.     

Proteus syndrome review: molecular,clinical, and pathologic features

Proteus syndrome is caused by an activating AKT1 mutation (c.49G>A, p.Glu17Lys). Many variable features are possible in this mosaic disorder, including: (i) disproportionate, asymmetric, and distorting overgrowth; (ii) bone abnormalities different from those observed in other disorders; (iii) a characteristic cerebriform connective tissue nevus made up of highly collagenized connective tissue; (iv) epidermal nevi in early life, consisting of acanthosis and hyperkeratosis; (v) vascular malformations of the capillary, venous, or lymphatic types; (vi) dysregulated adipose tissue including lipomas, lipohypoplasia, fatty overgrowth, and localized fat deposits; (vii) other unusual features, including bullous lung alterations; specific neoplasms; a facial phenotype associated with intellectual disability and/or seizures, and/or brain malformations; and (viii) deep vein thrombosis, resulting in premature death. Concluding remarks address diagnostic criteria, natural history, management, psychosocial issues, and differential diagnosis.     

Preliminary analysis of mortality associated with rituximab use in autoimmune diseases

Abstract

Normal antibodies and pathogenic autoantibodies are produced by B-cells and plasma cells. Rituximab is a chimeric monoclonal antibody that targets the CD20 molecule on cells that express them on their surface and kills them. Rituximab has been increasingly used to treat several autoimmune diseases. Studies on fatal outcomes associated with rituximab therapy are lacking. A comprehensive and detailed analysis in which the multiple factors that could contribute to a fatal outcome in all the autoimmune diseases in which rituximab has been used would be cumbersome, lack uniformity and would prove difficult in making certain definitive conclusions and comparisons, but more importantly it would not allow to provide specific precautions and recommendations to prevent mortality. Hence, autoimmune mucocutaneous blistering diseases (AMBD) were used as model to study fatal outcomes in patients treated with rituximab between 2000 and 2013, using uniform 13 criteria. Fatal outcomes were found in 14 patients with autoimmune blistering diseases out of 134 patients (10.4%). Patients died due to infections (75%), gastrointestinal (17%) and cardiac events (8%). Causes of death were reported in 101 patients with other autoimmune diseases out of 4320 with a mortality rate of 2.4%. Among them, 44 patients (43.6%) died from infections. A statistical analysis of the data demonstrated that a statistically significant higher mortality rate was observed in patients with AMBD compared to patients with other autoimmune diseases. Similarly, a statistically significant higher rate of death due to infections was reported in patients with AMBD compared to patients with other autoimmune diseases. Use of systemic corticosteroids and immunosuppressive agents as concomitant therapy with rituximab enhanced immunosuppression. In many patients, B-cells were depleted for prolonged periods, even after clinical recovery was observed. Although its main action is depletion of B-cells, rituximab has a significant impact on the immune and inflammatory systems, directly and indirectly and thus enhances susceptibility to infection. These preliminary data suggests that physicians using rituximab to treat autoimmune diseases should monitor their patients closely, especially their B-cell levels until they return to normal, be vigilant for possible sources of infection, and be aware of potential fatal outcomes.     

T and B cells target identical regions of the non-collagenous domain 1 of type VII collagen in epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita (EBA) is a severe immunobullous disease and is caused by IgG against type VII collagen (Col VII) of anchoring fibrils. In this study, utilizing ELISA and immunoblot, 13/15 EBA sera but 0/20 bullous pemphigoid sera and 0/30 healthy control sera showed IgG reactivity with distinct recombinant subregions of the non-collagenous domain 1 (NC1) of Col VII. In two EBA patients, IgG titers against Col VII-NC1 were grossly correlated to clinical disease activity. Moreover, Col VII-reactive T cells were identified in a representative EBA patient which recognized identical subdomains of Col VII-NC1. These findings strongly suggest that (1) the Col VII-NC1 ELISA is a powerful tool for making the diagnosis of EBA, (2) Col VII-specific IgG grossly relates to disease activity and (3) IgG reactivity is associated with T cell recognition of identical subdomains of Col VII-NC1.     

Epidermolysis bullosa acquisita in childhood

This case report of an 11-year-old girl describes a juvenile form of epidermolysis bullosa acquisita, an autoimmune disease of IgG antibodies to basement membrane type 7 collagen. Our case illustrates an unusually severe, acute inflammatory presentation of this condition with prominent mucosal and constitutional features requiring admission to a paediatric burns unit. The treatment consisted of supportive topical and systemic agents, prednisolone and dapsone. She responded to dapsone alone and the course of the illness was uneventful.     

Examination and Treatment of Patients with Angle-Closure Glaucoma in Japan: Results of a Nationwide Survey

Purpose To assess the present state of examination procedures and treatments for angle-closure glaucoma (ACG) in Japan.Methods A nationwide questionnaire survey was conducted, targeting 1237 registered facilities to Japanese Ophthalmological Society.Results Valid replies were obtained from 754 of 1232 facilities (61.2%). Primary ACG comprised a small percentage of the glaucoma types diagnosed in outpatient examinations. More than 50% of the laser iridotomy (LI) procedures were prophylactic at 52.9% of the facilities. Gonioscopy was most frequently used for determining indications for prophylactic LI. Specular microscopy was always performed prior to LI in 7.8% of the facilities. Prophylactic LI resulted in complications at 20.5% of the surveyed facilities, although the incidence was low. The following residual complications were reported at 82.9% of the facilities when treating ACG attacks: cataract, persistent elevation of intraocular pressure, and bullous keratopathy. One-third of the facilities experienced inadequate treatment of an ACG attack by physicians other than ophthalmologists.Conclusions The incidence of LI-induced complications was higher in ACG eyes than in narrow-angle eyes. However, prophylactic LI sometimes results in persistent ocular complications. Jpn J Ophthalmol 2004;48:133–140 © Japanese Ophthalmological Society 2004