• Title/Summary/Keyword: Systemic Lupus Erythematosus

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A Case of Systemic Lupus Erythematosus in a Dog (개에 있어서 전신성 홍반성 낭창 일례)

  • 김주향;김기홍;고인경;이금종;나기정;양만표
    • Journal of Veterinary Clinics
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    • v.17 no.2
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    • pp.443-449
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    • 2000
  • A three-year-old male jindo with generalized skin lesions (including seborrhea, hyperk- eratosis, alopecia, papules, and ecchymoses), pruritus lymph node enlargement, and fever was brought to Veterinary Teaching Hospital, College of Veterinary Medicine, Chungbuk National Uni- versity. There were no laboratory findings for parasites and fungi in the hair and skin But, the com- plete blood counts (CBC) showed leukocytosis and severe cosinophilia, It was suspected to be an inflammatory and allergic dermatitis. Thus, prednisoIone (0.5 mg/kg PO, BID for 1 week) and ampi- cillin (10 mg/kg PO, BID for 1 week given. One week later, pruritus and ecchymoses were reduced. These treatments were repeated for 7 day again. Three months later, the dog was presented again due to the relapse and exacerbation of the clinical signs. The signs were as follows; severe pru- ritus, vesicobullous skin lesions, anorexia, emaciation, lameness, and welling of carpal joints that showed inflammatory skin lesion and draining of synovia-like fluid. The values of WBC counts were returned to normal ranges. In contrast, eosinophilia was still observed. Coombs test for patient RBC and serum were negative. Hypoalbuminemia (2.5g/dl) was shown by serum chemistry. The uri- nalysis revealed and presence of leukocytes. Luxation finding of right radial carpal joint by polyarthritis was shown in radiography of affected joints.Lupus eryhematosus(LE) cells also appeared in peripheral blood and synovial fluid of affected joints. Definitely, antinuclear antibody (ANA) of patient serum using feline peripheral blood mononuclear cells was detected by all immu- nofluorescence. Based on these findings such as sedum ANA-Positive. major signs (skin disease, non- erosive polyarthritis with soft tissue swelling. and proteinuria), minor signs (fever), and LE cell-pos- itive, a diagnosis of systemic lupus crythematosus (SLE) was mad\ulcorner in this dog. The dog with SLE was administered with Pre(2.0 mg/kg PO, BID for first 4 week and then QOD) to inhibit the production of autoantibodies and with ampicillin (10 mg/kg PO, BID for first 4 weeks and then QOD) to prevent the secondary infection. The condition of this dog was monitored every 2 weeks by physical examinations, radiography, CBC, serum chemistry and urinalyais. At 8th week of treat- ment, the state of SLE evaluated by physical examinations and laboratory findings was markedly improved except for proteinura.

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A 22-month-old Boy with Acute Glomerulonephritis Coexistent with Hemolytic Anemia and Idiopathic Thrombocytopenia

  • Park, Hye Won;Seo, Bo Seon;Jung, Su Jin;Lee, Jun Ho
    • Childhood Kidney Diseases
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    • v.19 no.1
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    • pp.43-47
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    • 2015
  • Hemolytic anemia and thrombocytopenia are rare clinical manifestations of acute glomerulonephritis. Initially, in all such cases, a diagnosis of hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, systemic lupus erythematosus, and amyloidosis should be ruled out. The presence of hemolytic anemia and thrombocytopenia is rare, but possible, in a case of acute poststreptococcal glomerulonephritis, and may result in delayed diagnosis or misdiagnosis. Correct and timely diagnosis would ensure adequate treatment in such patients. We report of a 22-month-old boy with acute glomerulonephritis coexistent with hemolytic anemia and idiopathic thrombocytopenia.

Role of inflammasomes in inflammatory autoimmune rheumatic diseases

  • Yi, Young-Su
    • The Korean Journal of Physiology and Pharmacology
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    • v.22 no.1
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    • pp.1-15
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    • 2018
  • Inflammasomes are intracellular multiprotein complexes that coordinate anti-pathogenic host defense during inflammatory responses in myeloid cells, especially macrophages. Inflammasome activation leads to activation of caspase-1, resulting in the induction of pyroptosis and the secretion of pro-inflammatory cytokines including interleukin $(IL)-1{\beta}$ and IL-18. Although the inflammatory response is an innate host defense mechanism, chronic inflammation is the main cause of rheumatic diseases, such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), ankylosing spondylitis (AS), and $Sj{\ddot{o}}gren^{\prime}s$ syndrome (SS). Since rheumatic diseases are inflammatory/autoimmune disorders, it is reasonable to hypothesize that inflammasomes activated during the inflammatory response play a pivotal role in development and progression of these diseases. Indeed, previous studies have provided important observations that inflammasomes are actively involved in the pathogenesis of inflammatory/autoimmune rheumatic diseases. In this review, we summarize the current knowledge on several types of inflammasomes during macrophage-mediated inflammatory responses and discuss recent research regarding the role of inflammasomes in the pathogenesis of inflammatory/autoimmune rheumatic diseases. This avenue of research could provide new insights for the development of promising therapeutics to treat inflammatory/autoimmune rheumatic diseases.

Hidden Hematologic Disease in Trauma Patients: A Report of Two Cases

  • Jang, Sung Woo;Jung, Pil Young
    • Journal of Trauma and Injury
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    • v.33 no.2
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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.

Complement regulation: physiology and disease relevance

  • Cho, Heeyeon
    • Clinical and Experimental Pediatrics
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    • v.58 no.7
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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.

Cellular and Molecular Links between Autoimmunity and Lipid Metabolism

  • Ryu, Heeju;Kim, Jiyeon;Kim, Daehong;Lee, Jeong-Eun;Chung, Yeonseok
    • Molecules and Cells
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    • v.42 no.11
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    • pp.747-754
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    • 2019
  • The incidence of atherosclerosis is higher among patients with several autoimmune diseases such as psoriasis, rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). It is well documented that innate immune cells including macrophages and dendritic cells sense lipid species such as saturated fatty acids and oxidized low-density lipoprotein and produce pro-inflammatory cytokines and chemokines. However, whether a hyperlipidemic environment also impacts autoimmune T cell responses has been unclear. Among $CD4^+$ T cells, Th17 and follicular helper T (Tfh) cells are known to play pathogenic roles in the development of hyperlipidemia-associated autoimmune diseases. This review gives an overview of the cellular and molecular mechanisms by which dysregulated lipid metabolism impacts the pathogenesis of autoimmune diseases, with specific emphasis on Th17 and Tfh cells.

Clinical Manifestation and Treatment Outcome of Lupus Nephritis in Children (소아 루프스 신염의 임상양상 및 치료결과)

  • Park Jee-Min;Shin Jae-Il;Kim Pyung-Kil;Lee Jae-Seung
    • Childhood Kidney Diseases
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    • v.6 no.2
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    • pp.155-168
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    • 2002
  • Purpose; Systemic lupus erythematosus(SLE) is an autoimmune disease with multi-system involvement and renal damage is a major cause of morbidity and mortality in children. Renal involvement is more common and severe in children than in adults. Therefore, renal biopsy plays a crucial role in planning effective therapy. In this study, we investigated the clinical and pathological findings of lupus nephritis in children to aid clinical care of the disease. Methods: The clinical and pathological data of 40 patients who were diagnosed as SLE with renal involvement in Shinchon Severance Hospital from Jan. 1990 to Sep. 2002 were analyzed retrospectively. Results: The ratio of male to female patients was 1:3 and the median age at diagnosis was 12.1(2-18) years old. FANA(95.0%), anti-ds DNA antibody(87.5%), malar rash(80.0%) were the most common findings among the classification criteria by ARA. Microscopic hematuria with proteinuria(75.0%), nephrotic syndrome(55.0%), and microscopic hematuria alone(15.0%) were the most common renal presentations in the respective order at diagnosis. There were 27 cases with WHO class IV lupus nephritis confirmed by renal biopsy and 3 cases with pathological changes of WHO class type. Different treatment modalities were carried out : prednisolone only in 5 cases, prednisol-one+azat-hioprine in 9 cases, prednisolone+azathioprine+intravenous cyclophosphamide in 14 cases, prednisolone+cyclosporine A+intravenous cyclophosphamide in 12 cases, plasma exchange in 9 cases and intravenous gamma-globulin in 2 cases. The average follow-up period was $51.8{\pm}40.5$ months. During $51.8{\pm}40.5$ months. During follow-up, 4 patients expired. The risk factors associated with mortality were male, WHO class IV and acute renal failure at diagnosis. Conclusion: Renal involvement was noted in 63.5% of childhood SLE, and 67.5% of renal lesion was WHO class IV lupus nephritis which is known to be associated with a poor prognosis. Therefore aggressive treatment employing immunosuppressant during the early stages of disease could be helpful in improving long-term prognosis. But careful attention should be given to optimize the treatment due to unique problems associated with growth, psychosocial development and gonadal toxicity, especially in children.

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A Case of Sj$\ddot{o}$gren's Syndrome with Multiple Bullae Secondary to Pulmonary Amyloidosis and Lymphocytic Infiltration of Interstitium and Bronchioles (폐 유전분증(Amyloidosis)과 다발성 수 (Bullae), 폐 간질내 임파구 침윤이 동반된 Sj$\ddot{o}$gren's 증후군 1예)

  • Kim, Dong-Il;Lim, Yun-Jeong;Oh, Yung-Ha;Kim, Hyung-Soo;Lee, Jin-Sung;Kim, Dong-Soon
    • Tuberculosis and Respiratory Diseases
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    • v.44 no.6
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    • pp.1426-1432
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    • 1997
  • Sj$\ddot{o}$gren's syndrome(SS) is a chronic inflammatory disorder characterized by lymphocytic infiltration of lacrimal and sailvary glands, which results in dry eyes and dry mouth. SS may exist as a primary condition or as a secondary condition in association with connective tissue disease such as rheumatoid arthritis, systemic lupus erythematosus, or progressive systemic sclerosis. We experienced a patient with primary SS who developed multiple bullae, nodular type of pulmonary amyloidosis and lymphocytic interstitial peumonitis. We believe this to be the first reported case of SS acompanied by these three types pulmonary manifestations at the same time.

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Non-cirrhotic portal hypertension in an ankylosing spondylitis patient

  • Park, Sukki;Lee, Ji Hyun;Choi, Joon Sul;Kim, Hyun Woo;Shim, Beom Jin;Choi, Won Kyu;Kim, Sang Hyun
    • Journal of Yeungnam Medical Science
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    • v.35 no.1
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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.

The study on Fatigue, Pain, and Efficacy on Pain of Chronic Arthritis and Lupus (만성관절염과 루푸스의 피로, 통증, 및 통증효능감에 관한 연구)

  • Lim, Nan-Young;Lee, Eun-Young;Yang, Yong-Sook;Chung, Soon-Ae;Cha, Gyeong-Ok;Yi, Yeo-Jin
    • Journal of muscle and joint health
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    • v.7 no.1
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    • pp.53-62
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    • 2000
  • This study was designed to distinguish by the characteristic difference and the degree of symptoms such as fatigue, pain, coping to pain, and efficacy on pain, and to offer descriptive data for nursing intervention for improving coping ability to pain along each characteristics of disease in chronic arthritis and systemic lupus erythematosus. The subjects were 135 outpatients in the hospital for rheumatic disease in H-university, Seoul. The data were collected by structural questionnaire, from April 29 to June 29, 1999. The results were that the fatigue score was high in the OA patients while the RA patients and SLE patients experienced middle range of fatigue score, but which was not statistically different. Although the RA patients felt higher pain than other diseases, they have well coped with their pain than the others. In efficacy on pain the SLE patients had higher score than others but all of the disease showed lower score. No statistically significant difference among the three group was recorded in efficacy on pain. Therefore, pain management in the RA patients was primary nursing intervention because they felt severe pain and have well coped with pain while they had lower pain efficacy score than the others. It is also important that fatigue management and coping strategies on pain for the OA patients and SLE patients are specially supportive in the nursing intervention.

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