• Title/Summary/Keyword: Lupus erythematosus

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A Case of Hemophagocytic Lymphohistiocytosis in a Child with Systemic Lupus Erythematosus (전신성 홍반성 루푸스 환아에서 병발한 혈구 탐식성 조직구 증식증 1례)

  • Hwang, Ja Young;No, Suk Man;Lee, Jin;Jang, Pil Sang;Kim, Young Hoon;Kim, Jin Tack;Lee, Joon Sung
    • Clinical and Experimental Pediatrics
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    • v.46 no.10
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    • pp.1029-1031
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    • 2003
  • Hemophagocytic lymphohistiocytosis is a reactive disorder characterized by a generalized non-malignant histiocytic proliferation with prominent hemophagocytosis by stimulated histiocytes in the bone marrow and reticuloendothelial systems resulting in pancytopenia and liver dysfunction. Several diseases including infection, malignancy and autoimmune disease are known to be causative disorders. This case demonstrated histiocytic hemophagocytosis in the bone marrow, resulting in pancytopenia during treatment of systemic lupus erythematosus and did not show any underlying disease.

Two Cases of Thrombotic Thrombocytopenic Purpura in Systemic Lupus Erythematosus (전신성 홍반성 루프스와 동시 발병한 혈전성 혈소판 감소성 자반증 2예)

  • Kim, Hye-Young;Kim, Hyung-Hoi;Kim, Su-Yung
    • Childhood Kidney Diseases
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    • v.11 no.2
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    • pp.288-293
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    • 2007
  • Thrombotic thrombocytopenic purpura(TTP) is a rare but life-threatening multi-system disorder characterized by the classic pentad of clinical features that includes fever, microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities and renal dysfunction. TTP has been rarely reported to simultaneously present with systemic lupus erythematosus (SLE). While it is important to distinguish between the two diseases of therapeutic implication, cases of concurrent TTP and SLE help to elucidate the pathophysiology that underlies each condition. We describe two adolescents with synchronous TTP and SLE, and review the literature.

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Discoid lupus erythematosus (DLE) in a juvenile Alaskan Malamute dog

  • Kim, Ha-Jung;Yoo, Jong-Hyun;Kim, Ju-Won;Park, Chul;Lim, Chae-Young;Lee, Jong-Hwan;Quan, Fu-Shi;Park, Hee-Myung
    • Korean Journal of Veterinary Research
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    • v.49 no.1
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    • pp.73-78
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    • 2009
  • Discoid lupus erythematosus (DLE) is an immune-mediated skin disease which requires histopathology and immunohistopathology in both dogs and humans. A 10-month-old, intact female Alaskan Malamute presented for depigmentation, swelling, alopecia, erythema, and crusting on the bridge of the nose and the nasal planum. Cytological examination of nasal lesions revealed numerous cocci and neutrophils. Histopathological features included of infiltration of mononuclear cells at the dermoepidermal junction. Direct immunofluorescence tests and immunohistochemistry exhibited positive IgG, IgM, IgA, CD3, CD18, and CD79a on the epidermal basement membranes and around adnexal glands. This case indicates both T cells and B cells are related to mechanism of canine DLE. This case report describes advanced diagnostic tests and clinical outcome with immune suppressive therapy in a rare juvenile canine DLE case.

Recapitulation of Candidate Systemic Lupus Erythematosus-Associated Variants in Koreans

  • Kwon, Ki-Sung;Cho, Hye-Young;Chung, Yeun-Jun
    • Genomics & Informatics
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    • v.14 no.3
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    • pp.85-89
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    • 2016
  • Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organ systems. Although the etiology of SLE remains unclear, it is widely accepted that genetic factors could be involved in its pathogenesis. A number of genome-wide association studies (GWASs) have identified novel single-nucleotide polymorphisms (SNPs) associated with the risk of SLE in diverse populations. However, not all the SNP candidates identified from non-Asian populations have been validated in Koreans. In this study, we aimed to replicate the SNPs that were recently discovered in the GWAS; these SNPs have not been validated in Koreans or have only been replicated in Koreans with an insufficient sample size to conclude any association. For this, we selected five SNPs (rs1801274 in FCGR2A and rs2286672 in PLD2, rs887369 in CXorf21, rs9782955 in LYST, and rs3794060 in NADSYN1). Through the replication study with 656 cases and 622 controls, rs1801274 in FCGR2A was found to be significantly associated with SLE in Koreans (odds ratio, 1.26, 95% confidence interval, 1.06 to 1.50; p = 0.01 in allelic model). This association was also significant in two other models (dominant and recessive). The other four SNPs did not show a significant association. Our data support that FCGR polymorphisms play important roles in the susceptibility to SLE in diverse populations, including Koreans.

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

  • Kim, Jung-Hyun;Hah, Jung-Sang;Park, Mee-Young;Lee, Se-Jin;Lee, Jun
    • Journal of Yeungnam Medical Science
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    • v.23 no.1
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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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    • v.21 no.3
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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.

Systemic lupus erythematosus (전신성 홍반성 루푸스)

  • Kim, Kwang-Nam
    • Clinical and Experimental Pediatrics
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    • v.50 no.12
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    • pp.1180-1187
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    • 2007
  • Systemic lupus erythematosus (SLE) is an episodic, multi-system, autoimmune disease characterized by widespread inflammation of blood vessels and connective tissues and by the presence of antinuclear antibodies (ANAs), especially antibodies to native (double-stranded) DNA (dsDNA). Its clinical manifestations are extremely variable, and its natural history is unpredictable. Untreated, SLE is often progressive and has a significant fatality rate. The most widely used criteria for the classification of SLE are those of the American College of Rheumatology (ACR), which were revised in 1982 and modified in 1997. The presence of four criteria have been diagnosed as a SLE. Rashes are common at onset and during active disease. The oral mucosa is the site of ulceration with SLE. Arthralgia and arthritis affect most children and these symptoms are short in duration and can be migratory. Lupus nephritis may be more frequent and of greater severity in children than in adults. The initial manifestation of nephritis is microscopic hematuria, followed by proteinuria. The most common neuropsychiatric symptoms are depression, psychosis(hallucination and paranoia) and headache. CNS disease is a major cause of morbidity and mortality. Pericarditis is the most common cardiac manifestation. Libman-Sacks endocarditis is less common in children. The most frequently described pleuropulmonary manifestations are pleural effusions, pleuritis, pneunonitis and pulmonary hemorrhage. During the active phase ESR, CRP, gamma globulin, ferritin and anti-dsDNA are elevated. Antibodies to dsDNA occur in children with active nephritis. Antibodies to the extractable nuclear antigens (Sm, Ro/SS-A, La/SS-B) are strongly associated with SLE. Specific treatment should be individualized and based on the severity of the disease. Sepsis has replaced renal failure as the most common cause of death.

Eight Constitution Acupuncture Effect on Thrombocytopenia After Splenectomy at Idiopathic Thromobocytopenic Purpura as First Manifestation of Systemic Lupus Erythematosus Lupus (SLE환자에서 ITP 진단으로 비장적출술 후 혈소판감소를 팔체질침으로 호전시킨 치험례)

  • Seo, Yong-Won;Ko, Heung
    • Journal of Acupuncture Research
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    • v.23 no.4
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    • pp.219-224
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    • 2006
  • Idiopathic thrombocytopenic purpura(ITP) is characterized by the development of a specific anti-platelet autoantibody immune response mediating the development of thrombocytopenia. Systemic lupus erythematosus(SLE) is an autoimmune disease characterized by the production of a wide variety of autoantibodies. We experienced SLE patient whose initial symptoms were related to idiopathic thrombocytopenic purpura(ITP). She has a thrombocytopenia after Splenectomy and Steroid therapy on ITP and SLE. After she took Eight constitution Acupuncture treatment, thrombocytopenia has improved. We think Acupuncture will be effective treatment at autoimmune disease.

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Extent of Knowledge on the Disease and Learning Needs of Patients with Systemic Lupus Erythematosus (전신성 홍반성 낭창환자의 질병관련 지식정도와 교육 요구도)

  • Song Byung-Eun;You So-Yeon
    • Journal of Korean Academy of Fundamentals of Nursing
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    • v.6 no.3
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    • pp.464-476
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    • 1999
  • This study was conducted between October 1, 1998, and December 31, 1998, in order to provide basic data to develop better self-management educational programs for systemic lupus erythematosus (lupus) patients. In the study, the extent of each patients knowledge on the disease and their learning needs about it were examined for 100 lupus inpatients and outpatients at the Kangnam St. Marys Hospital. Data were collected by using questionnaires to those patients. Frequencies, percentage, average, standard deviation, t-test, ANOVA, and Duncans multiple range test were examined through the SAS program. The result of study follows : 1) The average score of patients knowledge on the disease was 15.7 (range, 7-20; standard deviation, 2.74). Subjects scored higher as far as the extent of their knowledge in the following categories : management of daily life, diet, characteristics of the disease, risk factors, and medication. Two categories shared the highest percentage of correct answers : 99% of subjects correctly identified that fatigue and stress aggravate symptoms of lupus and symptoms of lupus vary among individuals in range and type. However, when subjects were asked if patients should get a regular examination by an ophthalmologist every 4-6 months while they are on medication to treat rashes, lupus can be completely cured, and contraceptives that include a female hormone (estrogen) are good as contraceptive methods, the percentages of correct answers were low (32%, 31%, and 20%, respectively). In terms of subjects knowledge about the disease by their general characteristics, single subjects had more knowledge about the disease than married subjects did (t=2.14, p=0.0353). The extent of knowledge also varied by monthly income (F=4.96. p=0.0097). Those with more formal education had more knowledge about lupus than those who had less formation education did (t=2.95, 0.0039). Additionally, those who were satisfied with their education about the disease had better knowledge about it than those who were dissatisfied with their education did (t=2.71, p=0.0090). 2) The extent to which lupus patients wanted to be educated about the disease was, on average, 64.5 (range, 46-75; standard deviation, 7.91). Areas for which patients requested education are listed here in order : risk factors, sexual lives and patients associations. Patients who had been hospitalized demonstrated wanting more education than those who had not been did(t=3.73, p=0.0003). The extent to which they wanted the information was different by the number of educational sessions they had (F=3.98, p=0.0249). In conclusion, the results above would be considered when the education programme is planned for SLE patients.

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The Outcomes of an e-Wellness Program for Lupus Patients in Thailand: A Participatory Action Research Approach

  • Nop T. Ratanasiripong;Steven Cahill;Christine Crane;Paul Ratanasiripong
    • Journal of Preventive Medicine and Public Health
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    • v.56 no.2
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    • pp.154-163
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    • 2023
  • Objectives: Systemic lupus erythematosus (SLE) or lupus patients usually experience various physical and psychological challenges. Since the coronavirus disease 2019 pandemic, these challenges have become even harsher. Using the participatory action research approach, this study evaluated how an e-wellness program (eWP) impacted SLE-related knowledge and health behaviors, mental health, and quality of life among lupus patients in Thailand. Methods: A 1-group, pretest-posttest design study was conducted among a purposive sample of lupus patients who were members of Thai SLE Foundation. The 2 main intervention components were: (1) online social support, and (2) lifestyle and stress management workshops. Sixty-eight participants completed all the study requirements, including the Physical and Psychosocial Health Assessment questionnaire. Results: After being in the eWP for 3 months, participants' mean score for SLE-related knowledge increased significantly (t=5.3, p<0.001). The increase in sleep hours was statistically significant (Z=-3.1, p<0.01), with the percentage of participants who slept less than 7 hours decreasing from 52.9% to 29.0%. The percentage of participants reporting sun exposure decreased from 17.7% to 8.8%. The participants also reported significantly lower stress (t(66)=-4.4, p<0.001) and anxiety (t(67)=-2.9, p=0.005). The post-eWP quality of life scores for the pain, planning, intimate relationship, burden to others, emotional health, and fatigue domains also improved significantly (p<0.05). Conclusions: The overall outcomes showed promising results of improved self-care knowledge, health behaviors, mental health status, and quality of life. It is recommended that the SLE Foundation continues to use the eWP model to help the lupus patient community.