• Title/Summary/Keyword: Autoimmune lupus

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항인지질항체증후군을 동반하지 않은 일과성 단안 실명으로 발현된 전신성 홍반성 루푸스 1 예 (A Case of Systemic Lupus Erythematosus Presenting with Amaurosis Fugax without Antiphospholipid Antibodies Syndrome)

  • 김정현;하정상;박미영;이세진;이준
    • Journal of Yeungnam Medical Science
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    • 제23권1호
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    • pp.113-117
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    • 2006
  • Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that may affect many organ systems including the nervous system. The immune response in patients with SLE can cause inflammation and other damage that can cause significant injury to the arteries and tissues. A 48-year-old woman was admitted to the hospital because of transient monocular blindness. Magnetic resonance imaging and conventional angiography showed severe stenosis of the distal intracranial internal carotid artery. The patient was diagnosed as having SLE but the antiphospholipid antibodies were negative. Amaurosis fugax has not been previously reported as an initial manifestation of SLE in Korea. We report a patient with a retinal transient ischemic attack as the first manifestation of SLE.

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Identifying pathogenic variants related to systemic lupus erythematosus by integrating genomic databases and a bioinformatic approach

  • Ratih Dewi Yudhani;Dyonisa Nasirochmi Pakha;Suyatmi Suyatmi;Lalu Muhammad Irham
    • Genomics & Informatics
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    • 제21권3호
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    • pp.37.1-37.11
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    • 2023
  • Systemic lupus erythematosus (SLE) is an inflammatory-autoimmune disease with a complex multi-organ pathogenesis, and it is known to be associated with significant morbidity and mortality. Various genetic, immunological, endocrine, and environmental factors contribute to SLE. Genomic variants have been identified as potential contributors to SLE susceptibility across multiple continents. However, the specific pathogenic variants that drive SLE remain largely undefined. In this study, we sought to identify these pathogenic variants across various continents using genomic and bioinformatic-based methodologies. We found that the variants rs35677470, rs34536443, rs17849502, and rs13306575 are likely damaging in SLE. Furthermore, these four variants appear to affect the gene expression of NCF2, TYK2, and DNASE1L3 in whole blood tissue. Our findings suggest that these genomic variants warrant further research for validation in functional studies and clinical trials involving SLE patients. We conclude that the integration of genomic and bioinformatic-based databases could enhance our understanding of disease susceptibility, including that of SLE.

한방 변증과 양방 협진에 의한 전신성 홍반성 낭창(Systemic Lupus Erythematosus) 치료 1예 (One Case of Systemic Lupus Erythematosus treated by Integrated Therapy of Western Medicine with Oriental Differential Diagnosis of Symptoms and Signs)

  • 정대영;백동기;황상일;신선호;김동웅;한명아
    • 대한한방내과학회지
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    • 제23권2호
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    • pp.306-312
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    • 2002
  • Systemic Lupus Erythematosus(SLE) is a autoimmune disease characterized by combined symtoms of malar rash, discoid rash, neuropsychiatric disorder, renal disorder, hematologic disorder, photosensitivity immunologic disorder, oral ulcer, anti-nuclear antibody, arthritis, pleuritis and pericarditis, etc. Multiple genetic or environmental causes are supposed to facilitate antiboby production to autoantigen such as ds-DNA, histone, phospholipid, red blood cell, platelet, etc. And defective complementary system fail to remove autoantigen-antibody complex, which deposit in multiple organs and result in inflammatory damages. SLE does not correctly correspond to any specific category of oriental medicine. But, accoring to previous reports, it can be controlled by herb medications used differently patients-to-patients. So we are to report this one SLE case being successfully controlled by classic corticosteroids with herb medications based on oriental diffrential diagnosis of symptoms and signs.

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Enforced Expression of CXCR5 Drives T Follicular Regulatory-Like Features in Foxp3+ T Cells

  • Kim, Young Uk;Kim, Byung-Seok;Lim, Hoyong;Wetsel, Rick A.;Chung, Yeonseok
    • Biomolecules & Therapeutics
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    • 제25권2호
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    • pp.130-139
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    • 2017
  • $CXCR5^+$ T follicular helper (Tfh) cells are associated with aberrant autoantibody production in patients with antibody-mediated autoimmune diseases including lupus. Follicular regulatory T (Tfr) cells expressing CXCR5 and Bcl6 have been recently identified as a specialized subset of $Foxp3^+$ regulatory T (Treg) cells that control germinal center reactions. In this study, we show that retroviral transduction of CXCR5 gene in $Foxp3^+$ Treg cells induced a stable expression of functional CXCR5 on their surface. The Cxcr5-transduced Treg cells maintained the expression of Treg cell signature genes and the suppressive activity. The expression of CXCR5 as well as Foxp3 in the transduced Treg cells appeared to be stable in vivo in an adoptive transfer experiment. Moreover, Cxcr5-transduced Treg cells preferentially migrated toward the CXCL13 gradient, leading to an effective suppression of antibody production from B cells stimulated with Tfh cells. Therefore, our results demonstrate that enforced expression of CXCR5 onto Treg cells efficiently induces Tfr cell-like properties, which might be a promising cellular therapeutic approach for the treatment of antibody-mediated autoimmune diseases.

류마티스 관절염.루프스.섬유조직염 환자의 피로 양상 비교 (The Comparison of Fatigue of the Patients with Rheumatoid Arthritis, Lupus, and Fibromyalgia)

  • 이경숙;송경자;이은옥
    • 근관절건강학회지
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    • 제7권1호
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    • pp.131-147
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    • 2000
  • Almost all patients with rheumatic disease experience fatigue. The fatigue affects the patient's life extensively at home and at work, therefore it is necessary to investigate the nature of the fatigue which the patients perceive. The purpose of this study is to explore the nature and pattern of fatigue of the patients with rheumatic diseases. Rheumatoid arthritis is typical disease for its joint involvement which leads to deformity. Whereas lupus is a characteristic systemic autoimmune disease and the fibromyalgia is characterized by the general bodyache and multiple local tenderness. The prevalence of these diseases and the fatigue was known to be higher in women than men. Therefore the subjects were woman patient diagnosed as rheumatoid arthritis, lupus or fibromyalgia, and they were recruited from the H-Rheumatic Disease Hospital. The two instruments, the Multidimensional Assessment of Fatigue by Belza(1995) and the Piper Fatigue Scale by Piper, et al(1995) were used to explore the nature and pattern of self-reported fatigue. In total, the data from 157 patients were analysed by the SPSS-PC program for statistical analysis. The results were as follows: 1. Most patients with rheumatic disease experienced fatigue and the degree of fatigue was at the middle range by the scores of the two instruments. 2. The degree of fatigue of the patients with fibromyalgia was the highest and the next was that of the patients with lupus and the fatigue of the patients with rheumatoid arthritis. But there were no statistically significant differences among the patients with three rheumatic diseases, except the subcategory, the meaning of fatigue of the Piper Fatigue Scale. 3. Even when the period of the symptom and pain were covariated, there were no statistically significant differences among patients with three rheumatic diseases. The fatigue of the patients with lupus and fibromyalgia is rarely investigated in Korea and this study can be the base for the further understanding of the patients with rheumatic diseases. Therefore repeated studies are required to identify the factors to affect the fatigue and to understanding the nature of the diseases and to develop the nursing interventions to alleviate the fatigue.

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Th17과 자가면역 관절염 (The Th17 and Autoimmune Arthritis)

  • 조미라;허유정;박진실;이선영;성영철;김호연
    • IMMUNE NETWORK
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    • 제7권1호
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    • pp.10-17
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    • 2007
  • Autoimmune arthritis, such as rheumatoid arthritis (RA), is a chronic inflammatory disorder that primarily affects the joints and then results in their progressive destruction. Effector Th cells have been classified as Th1 and Th2 subsets based on their cytokine expression profiles and immune regulatory function. Another subset of T cells termed Th17 was recendy discovered and known to selectively produce IL-17. Also, Th17 was shown to be generated by TGF${\beta}$ and IL-6 and maintained by IL-23. IL-17 is a proinflammatory cytokine that is considered to involve the development of various inflammatory autoimmune diseases such as RA, asthma, lupus, and allograft rejection. IL-17 is present in the sera, synovial fluids and synovial biopsies of most RA patient. IL-17 activates RA synovial fibroblasts to synthesize IL-6, IL-8 and VEGF via PI3K/Akt and NF-${\kappa}B$ dependent pathway. IL-17 increases IL-6 production, collagen destruction and collagen synthesis. In addition, it not only causes bone resorption but also increases osteoclastogenesis and fetal cartilage destruction. Inhibition of the IL-17 production may contribute a novel therapeutic approach along with potent anti-inflammatory effect and with less immunosuppressive effect on host defenses.

자가면역 혈소판감소성 자반병과 관련된 항인지질 증후군 1례 (A Case of Antiphospholipid Syndrome Associated with Autoimmune Thrombocytopenic Purpura)

  • 남윤성;이우식;박찬;윤태기;차광열
    • Clinical and Experimental Reproductive Medicine
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    • 제26권2호
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    • pp.265-269
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    • 1999
  • Thrombocytopenic patients without detectable bound antiplatelet antibody should be diagnosed with idiopathic thrombocytopenic purpura (ITP) if no other cause of their decreased platelet count could be found. More recently the term "autoimmune thrombocytopenic purpura (ATP) has supplanted ITP since the disease is related to the production of autoantibodies against one's own platelets. This entity should not be confused with isoimmune thrombocytopenic purpura (also called alloimmune thrombocytopenic purpura). In this cases maternal antiplatelet antibodies directed against the PLA 1 antigen on the fetal platelets causes severe fetal and neonatal thrombocytopenia in a situation analogous to Rheusus disease. Antibodies to the negatively charged phospholipids, lupus anticoagulant, and anticardiolipin have been linked to adverse pregnancy events. Pregnant women possessing these antibodies have an increased risk of spontaneous abortion, stillbirths, intrauterine fetal growth retardation, preterm birth, and arterial and venous thrombosis. Antiphospholipid antibodies decrease or may even disappear between pregnancies only to recur with increased activity in a subsequent pregnancy and lead to loss. We have experienced a case of antiphospholipid syndrome associated with autoimmune thrombocytopenic purpura in patient with recurrent spontaneous abortion. So we report this case with a brief review of literatures.

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전신성 흥반성 낭창에 동반된 대동맥 판막 협착증의 수술 1에 (Aortic Stenosis in Systemic Lupus Erythematosus Syndrome)

  • 최주원;김우식;고행일;강윤경;김용인
    • Journal of Chest Surgery
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    • 제37권7호
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    • pp.613-616
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    • 2004
  • 전신성 홍반성 낭창은 자가면역질환의 일종으로 피부, 신장 및 심장에 빈번히 침범하는 질환으로 알려져 있으며, 심장에서는 심외막염, 심근염 및 심장 판막 질환을 발생시킬 수 있다. 전신성 홍반성 낭창에서의 심장 판막 질환은 주로 승모판막 및 대동맥 판막 폐쇄부전증인 것으로 알려져 있으며, 대동맥 판막의 협착증이 보고는 극히 적었다. 또한, 판막성 심장 질환이 발생하여도 다른 장기의 손상으로 인하여 수술을 받는 경우는 매우 드물며 국내보고도 확인되지 않았다. 저자들은 전신성 홍반성 낭창으로 받아오던 59세 여자 환자에게서 대동백 판막 협착증이 발생하였으며 이를 수술 치험하였기에 보고하는 바이다.

전신성 홍반성 루푸스 환자에서 발생한 괴사성 근막염의 치험례 (Necrotizing Fasciitis in a Patient with Systemic Lupus Erythematosus)

  • 조혜인;장학
    • Archives of Plastic Surgery
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    • 제38권3호
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    • pp.309-314
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    • 2011
  • Purpose: Necrotizing fasciitis is a life-threatening, destructive soft tissue infection with a very high rate of mortality that needs early diagnosis and aggressive treatment. Systemic Lupus Erythematosus (SLE) is a systemic, autoimmune disease and it's major cause of mortality is an infection. But necrotizing fasciitis in SLE is very rare and there have been only 22 cases reported in the literatures. We reported a patient of necrotizing fasciitis with SLE and reviewed 22 others from literature research. Methods: A 40-year-old female patient with a history of SLE for 6 years came to the emergency room. The patient complained of severe pain and swelling on her right leg. She was diagnosed as necrotizing fasciitis and underwent emergency fasciotomy. As wound cultures showed variable organisms, she was treated with broad-spectrum antibiotics and underwent several surgical debridements. Then, the wound was treated with the V.A.C (Vacuum Assisted Closure) device and split thickness skin grafting was performed two times. Results: Skin graft was well taken within 2 weeks after operations and the patient was discharged to outpatient follow up. There was no complication related with surgery and she could walk without cane after 3 months. Conclusion: We treated a necrotizing fasciitis in a patient with SLE and reviewed 22 others from literature research. The case presented here suggests that necrotizing fasciitis is a rare disease in SLE patients, but should be considered in the differential diagnosis of soft tissue infection in SLE patients. A high index of suspicion is needed for early diagnosis and proper management in these patients.

형제에서 발병한 가족성 전신 홍반 루푸스 (Familial systemic lupus erythematosus in two Korean male siblings)

  • 강현식;오현주;김영리;김재왕;신경수
    • Clinical and Experimental Pediatrics
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    • 제52권5호
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    • pp.611-614
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    • 2009
  • 전신 홍반 루푸스는 자가 항원에 대한 자가 항체를 생성하여 염증을 일으켜 다양한 기관에 손상을 주는 자가 면역 질환이다. 발병 원인은 잘 알려져 있지 않으나, 전신 홍반 루푸스 환자의 가족 중에 전신 홍반 루푸스가 일반인보다 20배 이상 발병 위험이 높아 유전적인 요인이 관련되어 있을 것으로 생각된다. 저자들은 형제에서 발병한 가족성 전신 홍반 루푸스 증례를 경험하였고, 전신 홍반 루푸스와 연관된 조직적합 유전자인 HLA DRB1*1501과 DQB1*0602 유전자를 환아모와 형제들이 공유한 것을 발견하였기에 문헌 고찰과 함께 보고하는 바이다.