• Annals of Clinical Neurophysiology
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• v.4 no.1
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• pp.1-6
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• 2002

The inflammatory myopathies are divided into three major and distinct subsets as polymyositis(PM), dermatomyositis(DM), and inclusion body myositis(IBM). This distinction is based on unique clinical, demographic, laboratory, histologic, therapeutic, prognostic, and immunopathologic criteria. Although the causes of PM, DM, and IBM are unknown, autoimmune mechanisms are implicated, as supported by their association with other putative or definite autoimmune diseases or viruses, the evidence for a T cell-mediated myocytotoxicity or complement-mediated microangiopathy, the presence of various autoantibodies and their response to immunotherapies. But in IBM the immune-mediated process is weaker and IBM patients do not readily respond to immunotherapies, there are convincing immunopathological signs to suggest that a definite autoimmune component, similar to that seen in PM, also plays a role in the cause of IBM.

• IMMUNE NETWORK
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• v.16 no.4
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• pp.219-232
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• 2016

Production of high affinity antibodies for antigens is a critical component for the immune system to fight off infectious pathogens. However, it could be detrimental to our body when the antigens that B cells recognize are of self-origin. Follicular helper T, or Tfh, cells are required for the generation of germinal center reactions, where high affinity antibody-producing B cells and memory B cells predominantly develop. As such, Tfh cells are considered as targets to prevent B cells from producing high affinity antibodies against self-antigens, when high affinity autoantibodies are responsible for immunopathologies in autoimmune disorders. This review article provides an overview of current understanding of Tfh cells and discusses it in the context of animal models of autoimmune diseases and allograft rejections for generation of novel therapeutic interventions.

• Clinical and Experimental Pediatrics
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• v.50 no.12
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• pp.1165-1172
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• 2007

Self/non-self discrimination and unresponsiveness to self is the fundamental properties of the immune system. Self-tolerance is a state in which the individual is incapable of developing an immune response to an individual's own antigens and it underlies the ability to remain tolerant of individual's own tissue components. Several mechanisms have been postulated to explain the tolerant state. They can be broadly classified into two groups: central tolerance and peripheral tolerance. Several mechanisms exist, some of which are shared between T cells and B cells. In central tolerance, the recognition of self-antigen by lymphocytes in bone marrow or thymus during development is required, resulting in receptor editing (revision), clonal deletion, anergy or generation of regulatory T cells. Not all self-reactive B or T cells are centrally purged from the repertoire. Additional mechanisms of peripheral tolerance are required, such as anergy, suppression, deletion or clonal ignorance. Tolerance is antigen specific. Generating and maintaining the self-tolerance for T cells and B cells are complex. Failure of self-tolerance results in immune responses against self-antigens. Such reactions are called autoimmunity and may give rise to autoimmune diseases. Development of autoimmune disease is affected by properties of the genes of the individual and the environment, both infectious and non-infectious. The host's genes affect its susceptibility to autoimmunity and the environmental factors promote the activation of self-reactive lymphocytes, developing the autoimmunity. The changes in participating antigens (epitope spreading), cells, cytokines or other inflammatory mediators contribute to the progress from initial activation to a chronic state of autoimmune diseases.

• IMMUNE NETWORK
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• v.11 no.6
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• pp.399-405
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• 2011

Background: Endogenous uveitis is a chronic inflammatory eye disease of human, which frequently leads to blindness. Experimental autoimmune uveoretinitis (EAU) is an animal disease model of human endogenous uveitis and can be induced in susceptible animals by immunization with retinal antigens. EAU resembles the key immunological characteristics of human disease in that both are $CD4^+$ T-cell mediated diseases. Dendritic cells (DCs) are specialized antigen-presenting cells that are uniquely capable of activating naive T cells. Regulation of immune responses through modulation of DCs has thus been tried extensively. Recently our group reported that donor strain-derived immature DC pretreatment successfully controlled the adverse immune response during allogeneic transplantation. Methods: EAU was induced by immunization with human interphotoreceptor retinoid-binding protein (IRBP) $peptide_{1-20}$. Dendritic cells were differentiated from bone marrow in the presence of recombinant GM-CSF. Results: In this study, we used paraformaldehyde-fixed bone marrow-derived DCs to maintain them in an immature state. Pretreatment with fixed immature DCs, but not fixed mature DCs, ameliorated the disease progression of EAU by inhibiting uveitogenic $CD4^+$ T cell activation and differentiation. Conclusion: Application of iBMDC prepared according to the protocol of this study would provide an important treatment modality for the autoimmune diseases and transplantation rejection.

• IMMUNE NETWORK
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• v.16 no.5
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• pp.271-280
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• 2016

Tertiary lymphoid structures (TLS) are ectopic lymphoid tissues involved in chronic inflammation, autoimmune diseases, transplant rejection and cancer. They exhibit almost all the characteristics of secondary lymphoid organs (SLO), which are associated with adaptive immune responses; as such, they contain organized B-cell follicles with germinal centers, distinct areas containing T cells and dendritic cells, high endothelial venules, and lymphatics. In this review, we briefly describe the formation of SLO, and describe the cellular subsets and molecular cues involved in the formation and maintenance of TLS. Finally, we discuss the associations of TLS with human diseases, especially autoimmune diseases, and the potential for therapeutic targeting.

• Tuberculosis and Respiratory Diseases
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• v.51 no.4
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• pp.390-394
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• 2001

Lymphocytic interstitial pneumonia(LIP) is characterized by a massive infiltration of the interstitium of the lung by mature lymphocytes, plasma cells and reticuloendothelial cells. LIP may be associated with autoimmune diseases including Sj$\check{o}$grens syndrome, SLE, myasthenia gravis, pernicious anemia, autoimmune hemolytic anemia, and HIV or an EB virus infection. There is a possibility of LIP progressing to a pulmonary or systemic lymphoma. The therapeutic response to corticosteroids and/or immunosuppressive drugs varies. Here we report a case of LIP that was diagnosed by an open lung biopsy and clonality study. The patient was a 36 year-old man without autoimmune disease or HIV infection. He was admitted as a result of severe hypoxemia showing $PaO_2$ of 48.3mmHg. The patient was treated with corticosteroids after the diagnosis and had fully recovered without a sequalae or relapse.

• IMMUNE NETWORK
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• v.4 no.3
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• pp.161-165
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• 2004

Background: Anti-cardiolipin antibody (Anti-CL Ab) is one of the various antiphospholipid antibodies (Anti-PL Abs) and found in the plasma of patients with systemic lupus erythematosus (SLE), atherosclerosis, and other infectious diseases. While anti-PL Abs found in the sera of patients with infectious diseases bind directly to CL, binding of anti-PL Abs to CL circulating in the sera of patients with autoimmune diseases is mediated by $\beta_2-$glycoprotein 1 ($\beta_2-GP1$). The purpose of this study is to investigate the effect of <$\beta_2-GP1$ on the antigen binding assay of anti-CL Abs present in the sera of patients with atherosclerosis, which has been known as one of autoimmune diseases. Methods: ELISA was performed with sera containing anti-CL Abs from three patients with atherosclerosis in the presence or absence of $\beta_2-GP1$ or FBS. Results: Reactivity of anti-CL Abs to CL was increased in the presence of $\beta_2-GP1$ or FBS in a dose dependent manner. Conclusion: <$\beta_2-GP1$ or FBS could be used as co-factor in CL ELISA with anti-CL Abs present in the sera of patients with atherosclerosis. It is suggested that anti-CL Abs found in atherosclerosis patients are similar in terms of antigen binding property to those circulating in the patients with autoimmune diseases, not to infectious diseases.

• Korean Journal of Veterinary Research
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• v.44 no.4
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• pp.483-486
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• 2004

The expression of nestin and vimentin in the spinal nerve roots of rats with experimental autoimmune encephalomyelitis (EAE) was studied to ascertain whether Schwann cells in the peripheral nerves respond to acute central nervous system autoimmune injury. Immunohistochemistry demonstrated that nestin was constitutively expressed in the dorsal roots of spinal nerves in control rats; its expression was enhanced in the spinal nerve roots of rats with EAE. Vimentin expression was weak in control rat spinal nerve roots, and it was increased in the dorsal roots of rats with EAE. It is postulated that normal animals have multipotent progenitor cells that constitutively express nestin and vimentin in the spinal nerve roots. In response to an injury of the central nervous system, these multipotent Schwann cells are activated in the spinal nerve roots through the expression of the intermediate filament proteins vimentin and nestin.

• Korean journal of dermatology
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• v.56 no.10
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• pp.620-623
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• 2018

Vitiligo is a multifactorial disorder. Neural, biochemical, and autoimmune mechanisms have been hypothetically suggested as etiopathological contributors to this condition. Autoimmunity focuses primarily on genetic factors and the association between vitiligo and other autoimmune disorders including autoimmune thyroid disease, rheumatoid arthritis, psoriasis, type 1 diabetes, pernicious anemia, and Addison's disease. We describe a 35-year-old man with systemic lupus erythematosus who developed concurrent vitiligo and discoid lupus erythematosus suggesting the possible autoimmune association between these 2 different diseases.

• Annals of Clinical Neurophysiology
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• v.8 no.1
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• pp.48-52
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• 2006

Background: Intravenous immunoglobulin (IVIg) has been administered for various immune-mediated neurological diseases such as autoimmune neuropathy, inflammatory myopathies, and other autoimmune neuromuscular disorders. The purpose of this study is to investigate side effects and complications of IVIg therapy in neuromuscular disorders. Methods: We enrolled 29 patients (age 8~63 years) with IVIg therapy for various neurological diseases including Guillain-Barre syndrome, myasthenia gravis, dermatomyositis, polymyositis, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. IVIg therapy was used at a dose of 0.4 g/kg body weight/day for 5 consecutive days. Results: 10 patients (34%) had adverse events. There are adverse events in 16 courses (11%) among total 145 courses. The majority of patients presented with mild side effects, mostly asymptomatic laboratory changes. Rash or mild headache occurred in 3 patients. One patient showed a serious side effect of deep vein thrombosis. Conclusions: IVIg therapy is safe for a variety of immune-mediated neurological diseases in our study.