简介：AbstractIntroduction:Numerous dermatoses associated with monoclonal gammopathy have been reported in the literature. Subepidermal autoimmune bullous diseases (SABD) are one of them which were not common.Here we report a case of SABD associated with MGUS who had a severe mucosal involvement and unexpected positive direct Nikolsky phenomenon.Case presentation:A 68-year-old male patient was admitted to our clinic with erosions on the oral mucosa, tense blisters, erosions, and ulcers on the trunk and extremities. Subepidermal vesicle formation was detected in the skin biopsy. Clinical examination revealed positivity for the Nikolsky phenomenon. The disease was unresponsive to conventional treatments and dysphagia and hoarseness occurred. The patient was screened for malignancy due to his unresponsiveness to the treatments and his severe oral mucosal involvement. Ig-G MGUS was detected in the patient.Discussion:The Nikolsky sign is an indicator of acantholysis and is known as a specific finding for pemphigus. However, when we look at gammopathy-associated autoimmune bullous dermatoses, skin fragility has been reported in cases. However, the meaning of fragility is not explained. The diagnosis of all these patients was Ig-M MGUS. Our patient was presented because of non-IgM MGUS, direct Nikolsky positivity, and severe mucosal involvement.Conclusion:Nikolsky positivity may be a clue for gammopathy-related subepidermal autoimmune bullous diseases.

简介：Highlevelsoflowmolecularweight(LMW)IgMincertaindiseasesareassociatedwithclinicalandlaboratoryindiceswhichreflecttheseverityofthedisease.TheseassociationssuggestthatLMWIgMmayplayanimportantroleintheimmunopathogenesisofthesediseases.TofurtherapproachthequestionconcerningthefunctionalactivityofLMWIgMindisease,apanelofLMWIgMandhighmolecularweight(HMW)IgMpreparationswithorwithoutrheumatoidfactor(RF)activitywereusedtoinvestigatetheirantibodybindingactivityandtheireffectorfunction.ItwasfoundthatLMWIgM-RFandHMWIgM-RFhadasimilarbindingcapacitytoFcfragmentastherewasnosignificantdifferenceintheaffinityindexbetweenthem.ItfurthershowedthattherateofactivationandtotalamountofutilizationofcomplementbyLMWIgMandHMWIgMwassimilar,althoughthemeanfluorescenceofC3depositionbyIgM-RFandHMWIgM-RFwasslightlyhigherthanthatofLMWIgM-RFandothercontrolRFantibodies.However,thecurrentstudydemonstratedthatLMWIgMhadstrongneutrophilactivatingpropertieswhencomparedwithHMWIgM.ThesefindingssuggestthatonemechanismofLMWIgMcontributingtotheimmunopathogenesisofRAmaybeduetotheformationofcirculatingimmunecomplex(CIC)byLMWIgMwithsubsequentactivationofneutrophils.WhetherLMWIgMhasotherfunctionalactivityindiseaseisunclearandneedsfurtherinvestigation.

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简介：AbstractThe complement system plays a key role in the pathogenesis of autoimmune diseases, which usually injures the kidney. More and more studies have shown the pathogenic role and indicated that abnormal activation of the complement system was highly involved in the outbreak of autoimmune diseases. This review mainly introduced recent studies of complement system activation contributing to the pathogenesis of autoimmune diseases, including systemic lupus erythematosus, antiphospholipid syndrome, antineutrophil cytoplasmic antibody-associated vasculitides, and so on. Understanding the pathogenic roles of complement activation in various autoimmune diseases will identify potential novel therapeutic targets on complement systems.

简介：因为他们的能力，B房间通常被认为是有免疫力的反应的积极管理者生产抗体，包括自身抗体。因为B房间用作介绍抗原的房间并且在有免疫力的回答施加另外的调节功能，抗体的生产便于最佳的CD4+T房间激活。然而，某些B房间能否定地也由生产规章的cytokines并且直接经由cell-to-cell接触与病原的T房间交往调整有免疫力的反应。B房间的这些类型被定义为规章的B(Breg)房间。Breg房间的规章的函数在发炎的鼠标模型被表明了，癌症，移植，并且特别地在autoimmunity。在这评论，我们集中于在人的自体免疫的疾病导致发展的理解和Breg房间的功能和B房间的含意的最近的进展。

简介：AbstractIntestinal homeostasis depends on complex interactions between the gut microbiota and host immune system. Emerging evidence indicates that the intestinal microbiota is a key player in autoimmune liver disease (AILD). Autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, and IgG4-related sclerosing cholangitis have been linked to gut dysbiosis. Diverse mechanisms contribute to disturbances in intestinal homeostasis in AILD. Bacterial translocation and molecular mimicry can lead to hepatic inflammation and immune activation. Additionally, the gut and liver are continuously exposed to microbial metabolic products, mediating variable effects on liver immune pathologies. Importantly, microbiota-specific or associated immune responses, either hepatic or systemic, are abnormal in AILD. Comprehensive knowledge about host-microbiota interactions, included but not limited to this review, facilitates novel clinical practice from a microbiome-based perspective. However, many challenges and controversies remain in the microbiota field of AILD, and there is an urgent need for future investigations.

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简介：ImmunizationwithinactivatedautoreactiveTcells(Tcellvaccination)selectedfromindividual'sownTcellrepertoireprovidesauniqueinvivosettingfortestingimmuneregulationthatisknowntoinvolveinteractionsofavarietyofrelatedsurfacemolecules(1).ItinducesregulatoryimmuneresponsesthatcloselyresembletheinvivosituationwheretheimmunesystemischallengedbyclonalactivationandexpansionofgivenTcellpopulationsinvariousautoimmunediseases.TcellvaccinationprovidesapowerfulmeansofelicitingnaturalreactionsoftheimmunesysteminresponsetoclonalexpansionofTcells,whichcanusedasatherapeuticapproachtosuppressoreliminatespecificpathogenicautoreactiveTcellsinautoimmuneconditions.ClinicaltrialsusingTcellvaccinationtodepleteautoreactiveTcellsinhumanautoimmuneconditionshavebeguntorevealthepathologicrelevanceofvariousautoimmuneTcellpopulationsinthediseaseprocesses,providingauniqueopportunitytotesttheautoimmunetheoriesinaclinicalsetting.Cellular&MolecularImmunology.2004;1(5):321-327.

简介：AbstractAutoimmune diseases with hematological manifestations are often characterized by chronicity and relapses despite treatment, and the underlying pathogenetic mechanisms remain unknown. Epigenetic alterations play a vital role in the deregulation of immune tolerance and the development of autoimmune diseases. In recent years, study of epigenetic mechanisms in both adult and childhood autoimmune disorders has been seeking to explain the pathophysiology of these heterogeneous diseases and to elucidate the interaction between genetic and environmental factors. Various mechanisms, including DNA methylation, histone modifications (chromatin remodeling), and noncoding RNAs (ncRNAs), have been studied extensively in the context of autoimmune diseases. This paper summarizes the epigenetic patterns in some of the most common childhood autoimmune disorders with hematological manifestations, based on epigenetic studies in children with primary immune thrombocytopenia (ITP), systemic lupus erythematosus (SLE), and juvenile idiopathic arthritis (JIA). Research findings indicate that methylation changes in genes expressed on T cells, modifications at a variety of histone sites, and alterations in the expression of several ncRNAs are involved in the pathogenesis of these diseases. These mechanisms not only determine the development of these diseases but also affect the severity of the clinical presentation and biochemical markers. Further studies will provide new tools for the prevention and diagnosis of childhood autoimmune disorders, and possible novel treatment options.

简介：AbstractAutoimmune diseases are primary immune diseases in which autoreactive antibodies or sensitized lymphocytes destroy and damage tissue and cellular components, resulting in tissue damage and organ dysfunction. Helper T cells may be involved in the pathogenesis of autoimmune diseases under certain conditions. This review summarizes recent research on the role of helper T cells in autoimmune diseases from two aspects, helper T cell-mediated production of autoantibodies by B cells and helper T cell-induced activation of abnormal lymphocytes, and provides ideas for the treatment of autoimmune diseases. The abnormal expression of helper T cells promotes the differentiation of B cells that produce autoantibodies, which leads to the development of different diseases. Among them, abnormal expression of Th2 cells and T follicular helper cells is more likely to cause antibody-mediated autoimmune diseases. In addition, abnormal activation of helper T cells also mediates autoimmune diseases through the production of abnormal cytokines and chemokines. Helper T cells play an essential role in the pathogenesis of autoimmune diseases, and a full understanding of their role in autoimmune diseases is helpful for providing ideas for the treatment of autoimmune diseases.

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简介：瞄准：为了评估磁性的回声cholangiopancreatography(MRCP)，与磁性的回声(先生)一起的调查结果在自体免疫的胰腺炎(AIP)想象病人。方法：有AIP的九个病人经历了MRI，MRCP，内视镜后退cholangiopancreatography(ERCP)，计算断层摄影术，和ultrasonography。在类固醇治疗前后拿的MRCP和先生图象被考察并且与另外的成像形式相比。AIP盒子的MRCP调查结果与有胰的头的癌的10个盒子的那些相比。结果：在MRCP上，在ERCP上注意的主要的胰腺的管的缩小的部分没被设想，当主要的胰腺的管的非包含的片断被设想时。近似的主要的胰腺的管的在上游的膨胀的度比在胰腺的癌的情况下看温和。更低的胆汁管的狭窄或阻塞在8个病人被检测。先生图象在T1加权的先生图象上与减少的信号紧张显示出胰的增大，T2加权的先生图象上的增加的信号紧张，并且，在3个病人，低亚硫酸钠强烈像囊的边界。在类固醇治疗以后，以前没设想主要的胰腺的管的部分被看见，与胆汁管狭窄的改进一起。胰腺的增大减少了，并且T1加权、T2加权的先生图象上的反常信号紧张成为了isointense。结论：MRCP不能区分从与胰腺的癌看见的主要的胰腺的管的狭窄与AIP看见的主要的胰腺的管的不规则的变窄。然而，与在T1加权、T2加权的先生图象上显示出反常信号紧张的胰腺的增大的先生成像一起的MRCP调查结果在支持AIP的诊断是有用的。

简介：Ltmedisease(Lymeborreliosis)iscausedbythetick-bornespiro-elraeteBorreliaburgdooCeri.Erythemamigrans,anearlyskinlesion,isthemostcommonclinicalpresentation.Theorganismmayspread,causingvariouslatermanifestations.

简介：春季是传染性疾病的高发时段，预防工作一定要做到及时、有效。也许从下面的短文中我们可以得到一些启示！

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