• Annals of Clinical Neurophysiology
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• 제7권2호
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• pp.93-96
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• 2005

Background: Myasthenia gravis (MG) and systemic lupus erythematosus (SLE) are well recognized to coexist and have some similarities in immunologic, clinical and serologic findings. Despite several reports of the association with autoantibodies and thymectomy in these disorders, the pathomechanism of coexistence remains to be elucidated. Objective: We aimed to investigate the relationship of MG and SLE through overall features of patients with both disorders;: clinical, laboratory, and electrophysiological findings. Materials and Methods: We reviewed the medical records of 6 consecutive patients with MG and SLE (2 men, 4 women, ages 17-51, mean 30.5 years, Seoul National University Hospital, from 1998 to 2005). Results: Three patients who developed SLE first, had ocular type of MG and 2 were children showing much severe and recurrent SLE features and only 1 patient had thymic hyperplasia. The other 3 developed MG first and they were generalized type and none underwent thymectomy. In addition, the development of MG or SLE was not coincident with remission or improvement of another disorder. Conclusion: The coexistence of SLE and MG may support the hypothesis of two different antibody populations modulated by thymus in the opposite extremesThis report suggests that the systemic and extensive autoimmune response in preceding MG or SLE may effect the development of the other disorder followed, while. the coexistence of two disorders cannot be explained by the hypothesis of two different antibody populations modulated by thymus in the opposite extremes The role of thymectomy and the theorectical subsequent effect on the development of SLE have been debated with controversy. However, SLE occurred without thymectomy in MG and these disorders did not develop in the quiescent period of another disorder. Therefore, the other pathomechanism for the coexistence of MG and SLE should be elucidated.

• 대한한의학회지
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• 제25권4호
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• pp.188-199
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• 2004

Transglutaminase 2 (TGase 2) is an enzyme that is widely used in many biological systems for generic tissue stabilization purposes or immediate defenses for wounds. Many reports have showed that TGase 2 is aberrantly activated in tissues and cells and contributes to a variety of diseases, including neurodegenerative diseases and autoimmune diseases. In most cases, the TGase 2 appears to be a factor in the formation of inappropriate proteinaceous aggregates that may be cytotoxic. However, in other cases such as celiac disease, arthritis, lupus, amyotrophic lateral sclerosis, TGase 2 is involved in the generation of autoantibodies. This suggests the possibility that the inappropriate expression and/or presentation of TGase 2 to T cells might contribute to these diseases in genetically predisposed individuals. Others and we have found that TGase 2 expression is also increased in the inflammation process. We also demonstrated reverse of inflammation by TGase inhibition. Furthermore we discovered the genuine role of TGase 2 in immune cell activation. Increase of TGase activity induces or exacerbates inflammation via NF-κB activation without I-κBα kinase signalings. This review will examine a possibility of TGase inhibitors as therapeutic agents in a variety of inflammatory diseases.

• Journal of Yeungnam Medical Science
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• 제35권1호
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• pp.89-93
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• 2018

Idiopathic non-cirrhotic portal hypertension (INCPH) is a disease with an uncertain etiology consisting of non-cirrhotic portal hypertension and portal pressure increase in the absence of liver cirrhosis. In INCPH, patients exhibit normal liver functions and structures. The factors associated with INCPH include the following: Umbilical/portal pyremia, bacterial diseases, prothrombic states, chronic exposure to arsenic, vinyl chloride monomers, genetic disorders, and autoimmune diseases. Approximately 70% of patients present a history of major variceal bleeding, and treatment relies on the prevention of complications related to portal hypertension. Autoimmune disorders associated with INCPH are mainly systemic sclerosis, systemic lupus erythematosus and rheumatoid arthritis. To the best of our knowledge, a case of ankylosing spondylitis (AS) associated with INCPH has not been reported thus far. Therfore, we report our experience of a patient with AS accompanied by INCPH, who showed perisplenic varices with patent spleno-portal axis and hepatic veins along with no evidence of cirrhosis on liver biopsy, and provide a brief literature review.

• Clinical and Experimental Pediatrics
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• 제50권1호
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• pp.74-78
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• 2007

목 적 : 전신성 홍반성 낭창(systemic lupus erythematosus, SLE)은 장기간에 걸쳐 다양한 임상경과를 나타내며 여러장기를 침범하는 자가 면역성 질환으로 유전적, 환경적, 면역학적 요인 등에 의한 면역 조절계의 이상으로 생각되고 있다. 소아에서는 갑작스럽게 발병하며, 경과가 빠르게 악화되며 여러 장기를 동시에 침범한다. 따라서 소아에서 조기 진단하여 조기 치료하는 것이 SLE의 예후 개선에 더욱 중요한 점으로 생각된다. 이에 저자들은 소아에서 발생한 SLE 환아의 임상 양상, 검사 소견, 예후에 대해 전반적으로 고찰하여 질환의 진단 및 예후의 향상을 기대하고자 한다. 방 법 : 1996년 1월부터 2005년 12월까지 10년간 신촌 세브란스 병원 소아과에서 전신성 홍반성 낭창으로 진단받은 45례의 환아를 대상으로 의무기록을 후향적으로 고찰하였다. 결 과 : 진단 당시 평균발병 연령은 $10.8{\pm}3.8$(0-15)세였으며 10-15세 사이에서 호발(68.9%)했으며 환아의 남녀비는 1:4였다. 진단시 초기증상으로 안면 부종(51.1%), 나비모양 홍반(44.4%), 발열(28.9%), 관절통(15.6%), 빈혈(13.3%) 및 광과민성(11.1%)순 이였다. SLE의 ARA 진단 기준중 항목별 양성률은 형광항핵항체(97.8%), 항ds DNA항체(82.2%), 루프스 신염(71.1%), 나비형 홍반(71.1%) 및 혈액학적 이상(66.7%)의 순이었다. 진단 당시 루프스 신염이 동반 되었던 경우는 45례 중 23례(51.1%)였으며 치료 중 8례가 루프스 신염으로 새로 진단되어 그 빈도가 71.1%로 조사되었고 신생검상 WHO class IV 병변인 미만성 증식성 사구체 신염(43.8%)이 가장 많았다. 2례는 완치되었으며, 2례 모두 신생아 루프스였다. 우리나라 아동에서 외국에 비하여 발열, 광과민성, 입궤양, 관절통, 늑막염은 적게 관찰되는 양상을 보였다. 결 론 : 소아에서 전신성 홍반성 낭창의 임상 양상과 예후는 다양하다. 장기적인 추적 관찰이 필요하며 예후의 향상을 위해 조기 진단과 적극적인 치료가 중요하며, 향후 이 질환에 대한 심도 있는 연구가 지속되어야 할 것이다.

• IMMUNE NETWORK
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• 제4권3호
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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.

• Journal of Trauma and Injury
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• 제33권2호
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• pp.112-118
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• 2020

In trauma patients, coagulopathy and abnormal increases or decreases in cell counts are frequently observed, and are associated with high mortality and morbidity in the acute phase of trauma. Because major trauma is often life-threatening, and hematologic abnormalities are multi-factorial and transient, major blood loss is usually suspected to be the primary cause of these abnormalities, and much time and cost may be spent attempting to identify a focus of hemorrhage that might or might not actually exist. Persistent abnormalities in the complete blood count, however, require clinical suspicion of other hematologic diseases to minimize improper transfusions and to improve outcomes, including mortality. Physicians at trauma centers should be familiar with the clinical characteristics of hematologic diseases and should consider these diseases in trauma patients. In this report, we present cases of two hematologic disorders found in trauma patients: autoimmune hemolytic anemia induced by systemic lupus erythematosus and myelodysplastic syndrome.

• Tuberculosis and Respiratory Diseases
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• 제74권4호
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• pp.151-162
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• 2013

Diffuse alveolar hemorrhage (DAH) is a life-threatening and medical emergency that can be caused by numerous disorders and presents with hemoptysis, anemia, and diffuse alveolar infiltrates. Early bronchoscopy with bronchoalveolar lavage is usually required to confirm the diagnosis and rule out infection. Most cases of DAH are caused by capillaritis associated with systemic autoimmune diseases such as anti-neutrophil cytoplasmic antibody-associated vasculitis, anti-glomerular basement membrane disease, and systemic lupus erythematosus, but DAH may also result from coagulation disorders, drugs, inhaled toxins, or transplantation. The diagnosis of DAH relies on clinical suspicion combined with laboratory, radiologic, and pathologic findings. Early recognition is crucial, because prompt diagnosis and treatment is necessary for survival. Corticosteroids and immunosuppressive agents remain the gold standard. In patients with DAH, biopsy of involved sites can help to identify the cause and to direct therapy. This article aims to provide a general review of the causes and clinical presentation of DAH and to recommend a diagnostic approach and a management plan for the most common causes.

• Clinical and Experimental Pediatrics
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• 제53권2호
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• pp.136-145
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• 2010

Natural killer T (NKT) cell is a special type of T lymphocytes that has both receptor of natural killer (NK) cell (NK1.1, CD161c) and T cell (TCR) and express a conserved or invariant T cell receptor called $V{\alpha}14J{\alpha}18$ in mice or Va24 in humans. Invariant NKT (iNKT) cell recognizes lipid antigen presented by CD1d molecules. Marine-sponge-derived glycolipid, ${\alpha}-galactosylceremide$ (${\alpha}-GalCer$), binds CD1d at the cell surface of antigen-presenting cells and is presented to iNKT cells. Within hours, iNKT cells become activated and start to secrete Interleukin-4 and $interferon-{\gamma}$. NKT cell prevents autoimmune diseases, such as type 1 diabetes, experimental allergic encephalomyelitis, systemic lupus erythematous, inflammatory colitis, and Graves' thyroiditis, by activation with ${\alpha}-GalCer$. In addition, NKT cell is associated with infectious diseases by mycobacteria, leshmania, and virus. Moreover NKT cell is associated with asthma, especially CD4+ iNKT cells. In this review, I will discuss the characteristics of NKT cell and the association with inflammatory diseases, especially asthma.

• Clinical and Experimental Pediatrics
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• 제58권7호
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• pp.239-244
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• 2015

The complement system is part of the innate immune response and as such defends against invading pathogens, removes immune complexes and damaged self-cells, aids organ regeneration, confers neuroprotection, and engages with the adaptive immune response via T and B cells. Complement activation can either benefit or harm the host organism; thus, the complement system must maintain a balance between activation on foreign or modified self surfaces and inhibition on intact host cells. Complement regulators are essential for maintaining this balance and are classified as soluble regulators, such as factor H, and membrane-bound regulators. Defective complement regulators can damage the host cell and result in the accumulation of immunological debris. Moreover, defective regulators are associated with several autoimmune diseases such as atypical hemolytic uremic syndrome, dense deposit disease, age-related macular degeneration, and systemic lupus erythematosus. Therefore, understanding the molecular mechanisms by which the complement system is regulated is important for the development of novel therapies for complement-associated diseases.

• Tuberculosis and Respiratory Diseases
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• 제52권1호
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• pp.76-85
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• 2002

BALT 림프종은 대부분 비호지킨스 림프종의 저등급의 B-세포 림프종으로서 쇼그렌씨 증후군 및 류마티스 관절염 등과 같은 자가면역질환과 관계가 있다고 알려져 있으나 전신성 홍반성 낭창에서 BALT 림프종의 발생에 대한 보고는 없는 것 같다. 저자들은 흉막성 통증을 주소로 내원한 54세 남자환자에서 전신성 홍반성 낭창으로 인한 흉막염의 원인규명과정에서 진단된 BALT 림프종에 대하여 문헌고찰과 함께 보고하는 바이다.