• Title/Summary/Keyword: Systemic Lupus Erythematosus

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자신활혈탕(滋腎活血湯)과 스테로이드 병합요법으로 관해를 보인 소아 낭창성 신염 환자 1예 (A Case Report of Lupus Nephritis in a Child Patient Treated with Jasinwhalhyul-tang and Steroid Therapy)

  • 장선규;최정식;안소현;조충식;김철중
    • 대한한방내과학회지
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    • 제29권3호
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    • pp.819-826
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    • 2008
  • In childhood systemic lupus erythematosus patients, renal involvement is closely related to mortality and morbidity of the disease. Therefore early diagnosis and treatment are essential to improving prognosis. We saw a child who had hematuria, albuminuria, anorexia, fatigue, and light hyper sensitiveness. He was diagnosed as lupus nephritis (WHO Class II+IV) and treated with ACE inhibitor and steroid therapy for 12 months. However, clinical improvement was not shown. So we treated him with herbal formula (Jasinwhalhyul-tang: Zishenhuoxue-tang) and steroid therapy. After 17 months of treatment, hematuria disappeared and clinical symptoms and albuminuria had improved significantly.

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병리조직검사에서 "Full-house" 패턴의 면역 복합체 침착이 발견된 비루푸스 신염 (A Case of "Full-house" Nephropathy in a Non-lupus Patient)

  • 유하영;손미경;조명현;곽병옥;박혜원;임소덕;정소정;김교순
    • Childhood Kidney Diseases
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    • 제18권2호
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    • pp.128-131
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    • 2014
  • "full-house" 면역 복합체 침착은 루푸스 신병증의 진단적인 조직 소견이다. 이 증례 보고에서 12세 남자 환아는 지속적인 현미경적 혈뇨와 단백뇨를 주소로 내원하였다. 그는 신장 조직 검사에서 "full-house" 신병증을 진단받았으나 전신성 홍반성 낭창과 관련한 어떠한 임상적 징후나 혈청학적인 결과를 보이지 않았다. 비록 "full-house" 신병증이 전신성 홍반성 낭창에 선행하는 질병인지에 대해서는 명확히 밝혀진 바는 없으나, 루푸스와 관련된 증상이나 혈청학적인 자가 항체가 출현할 수 있으므로, 추적 관찰을 요한다. 대부분의 현미경적 혈뇨는 좋은 예후를 가지고 있고, 보통은 소변검사를 정기적으로 검사하며 관찰한다. 그러나 이 증례에서 보듯이 소변검사 이상이 발견 되었을 때 오랫동안 무증상으로 잠복하는 "full-house" 신병증 또한 고려되어야 하며, 이는 몇몇의 임상적인 증상 및 혈청학적 소견이 음성이었던 환자가 전신성 홍반성 낭창으로 진행할 수 있기 때문이다.

TMEM39A and Human Diseases: A Brief Review

  • Tran, Quangdon;Park, Jisoo;Lee, Hyunji;Hong, Youngeun;Hong, Suntaek;Park, Sungjin;Park, Jongsun;Kim, Seon-Hwan
    • Toxicological Research
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    • 제33권3호
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    • pp.205-209
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    • 2017
  • Transmembrane Protein 39A (TMEM39A) is a member of TMEM family. The understanding about this protein is still limited. The earlier studies indicated that TMEM39A was a key mediator of autoimmune disease. TMEM39A seems to be involved in systemic lupus erythematosus and multiple sclerosis in numerous of populations. All of these works stop at insufficient information by using gene functioning methods such as: Genome-wide association studies (GWASs) and/or follow-up study. It is the fact that the less understood of TMEM39A actually is the attraction to the scientist in near future. In this review the current knowledge about TMEM39A and its possible roles in cell biology, physiology and pathology will be described.

루푸스 환자의 피로 관련 요인 (An Analytical Study on Fatigue and its Associated Variables In Patients with Systemic Lupus Erythematosus in Korea)

  • 송경애;강성실
    • 한국보건간호학회지
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    • 제16권1호
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    • pp.105-114
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    • 2002
  • This study was to investigate the level of fatigue and its associated variables in patients with systemic lupus erythematosus(SLE) in Korea. From March to June, 2001, 100 patients, registered at one university hospital rheumatology clinic in Seoul, were accepted as subjects for this study. The sampling method was adopted a non-probability, purposive technique. The instruments used for this study were The Multidimensional Assessment Fatigue scale developed by Tack and Beck Depression Instrument develped by Beck. The collected data were analyzed by SAS program using t-test, ANOVA with Scheffe-test, Pearson correlation coefficients and stepwise multiple regression. The results were as follows: 1. Total scores of fatigue of the subjects averaged $24.46(\pm10.85)$, degree of fatigue was $5.08(\pm2.29)$, and influence of fatigue was $3.52(\pm2.12)$. 2. Regarding characteristics, more depressive(p=.0001) and more painfuI(p=.0122) patients revealed more fatigue. Also, the subjects with spouse(p=.0337) and having poor quality of sleep(p=.0445) revealed more fatigue. 3. The subjects' total fatigue score, depression, pain and age was correlated positively(r=.53; r=.48; r=.24), and total fatigue score, and exercise time, quality of sleep was correlated negatively(r=-.45; r=-.21). 4. The main influencing factors on the fatigue were depression$(52.92\%)$ and quality of sleep $(8.10\%)$. These two main variables made it possible to explain $61.02\%$ of the varience in fatigue. In conclusion, this study revealed depression and quality of sleep is an important factor that can improve quality of life in patients with SLE. It is recommended that nursing intervention for SLE patients would be focused to decrease depression and to enhance quality of sleep.

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Dyslipidemia promotes germinal center reactions via IL-27

  • Ryu, Heeju;Chung, Yeonseok
    • BMB Reports
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    • 제51권8호
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    • pp.371-372
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    • 2018
  • Cardiovascular disease such as atherosclerosis is caused by imbalanced lipid metabolism and represents a leading cause of death worldwide. Epidemiological studies show that patients with systemic autoimmune diseases exhibit a higher incidence of atherosclerosis. Conversely, hyperlipidemia has been known to accelerate the incidence of autoimmune diseases in humans and in animal models. However, there is a considerable gap in our understanding of how atherosclerosis impacts the development of the autoimmunity in humans, and vice versa. The atherosclerosis-related autoimmune diseases include psoriasis, rheumatoid arthritis, systemic lupus erythematosus (SLE) and diabetes mellitus. By using animal models of atherosclerosis and SLE, we have recently demonstrated that hyperlipidemia significantly accelerates the development of autoantibodies, by inducing autoimmune follicular helper T ($T_{FH}$) cells. Mechanistic studies have identified that hyperlipidemia induces IL-27 production in a TLR4-dependent manner, likely via downregulating LXR expression in dendritic cells. In this case, mice lacking IL-27 do not develop enhanced antibody responses. Thus it is noted that these findings propose a mechanistic insight responsible for the tight association between cardiovascular diseases and SLE in humans.

전신성 홍반성 루푸스 산모의 출산아의 임상적 고찰 (완전 방실 블록을 중심으로) (Outcome of pregnant mothers with systemic lupus erythematosus (focusing on congenital heart block))

  • 백혜성;최재형;김남수;김창렬;문수지
    • Clinical and Experimental Pediatrics
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    • 제49권4호
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    • pp.381-387
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    • 2006
  • 목 적 : 신생아 루푸스는 전신성 홍반성 루푸스 산모의 자가 항체가 태반을 통해 태아에게로 넘어가 임상 증상을 보이는 질환으로 선천성 완전 방실 블록, 피부 병변, 간질환, 혈구 감소증을 특징으로 하는 질환이다. 이에 저자들은 전신성 홍반성 루푸스 산모의 출산아에서 선천성 완전 방실 블록의 빈도 및 출산아의 임상적 특징을 알아보고자 본 연구를 시행하였다. 방 법 : 1997년 1월부터 2005년 1월까지 한양대학교병원 류마티스 병원과 산부인과에서 진료받은 산모 55명의 57회의 임신결과와 태어난 49례의 신생아에 대한 임상기록을 후향적으로 검토하였다. 결 과 : 57례의 임신 중 5례(8.8%)의 자연유산, 1례(1.8%)의 사산이 있었고 15례(26.3%)의 조산, 8례(12.3%)의 부당 경량아의 빈도를 확인할 수 있었다. 57례의 임신 중 산전 초음파 검사에서 선천성 방실 블록 1례(1.8%)가 있었고 보호자 자의에 의해서 인공 유산이 행해졌다. 49례의 신생아 심전도 소견상 선천성 완전 방실 블록은 없었으며 모두 정상이었고 임상 기록상 피부병변이 있는 신생아는 없었다. 49례의 출산아 중 39례에서 혈액검사가 시행되었고 10례(25.6%)에서 혈액학적 이상이 있었다. 이 중 빈혈이 8례(20.5%), 중성구 감소증이 2례(5.1%), 혈소판 감소증이 3례(7.7%) 있었다. 자가 항체 anti-SSA(Ro)가 양성인 산모와 antiphospholipid 항체(aPL 항체)가 양성인 산모에서 미숙아 출산 빈도가 높았다(P=0.003, P=0.049). 항cardiolipin 항체(aCL 항체) 양성인 산모의 출산아가 인공환기요법을 받은 빈도가 높았다(P=0.018). 결 론 : 전신성 홍반성 루푸스 산모의 임신 중 선천성 완전 방실 블록의 빈도는 1.8%로 그 빈도가 낮았고 출산아 중 혈액학적 이상은 20% 이상에서 나타났다. 소홀히 하기 쉬운 피부병변에 대한 주의 깊은 추적 관찰로 신생아 홍반성 루푸스 진단에 관심을 가져야 할 것이다.

Diffuse Alveolar Hemorrhage

  • Park, Moo Suk
    • 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.

Aged Sanroque Mice Spontaneously Develop Sjögren's Syndrome-like Disease

  • Suk San Choi;Eunkyeong Jang;Yeon-Kyung Oh;Kiseok Jang;Mi-La Cho;Sung-Hwan Park;Jeehee Youn
    • IMMUNE NETWORK
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    • 제19권1호
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    • pp.7.1-7.11
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    • 2019
  • Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disorder that affects mainly salivary and lacrimal glands, but its cause remains largely unknown. Clinical data indicating that SS occurs in a substantial proportion of patients with lupus points to common pathogenic mechanisms underlying the two diseases. To address this idea, we asked whether SS develops in the lupus-prone mouse strain sanroque (SAN). Owing to hyper-activation of follicular helper T (Tfh) cells, female SAN mice developed lupus-like symptoms at approximately 20 wk of age but there were no signs of SS at that time. However, symptoms typical of SS were evident at approximately 40 wk of age, as judged by reduced saliva flow rate, sialadenitis, and IgG deposits in the salivary glands. Increases in serum titers of SS-related autoantibodies and numbers of autoantibody-secreting cells in cervical lymph nodes (LNs) preceded the pathologic manifestations of SS and were accompanied by expansion of Tfh cells and their downstream effector cells. Thus, our results suggest that chronic dysregulation of Tfh cells in salivary gland-draining LNs is sufficient to drive the development of SS in lupus-prone mice.

비세균성 신염환자에서 신장내 $^{67}Ga-Citrate$ 흡수에 관한 연구 (Renal Localization of Ga-67 Citrate in Noninfectious Nephritis)

  • 이강욱;정민수;이순구;김삼용;신영태;노흥규
    • 대한핵의학회지
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    • 제26권2호
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    • pp.318-326
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    • 1992
  • Ga-67 citrate scan has been requested for detection or follow-up of inflammatory or neoplastic disease. Visualization of Ga-67 citrate in the kidneys at 48 and 72 hr post injection is usually interpreted as evidence of renal pathology. But precise mechanisms of abnormal Ga-67 uptake in kidneys were unknown. We undertook a study to determine the clinical value of Ga-67 citrate imaging of the kidneys in 68 patients with primary or secondary nephropathy confirmed by renal biopsy and 66 control patients without renal disease. Renal uptake in 48 to 72 hr images was graded as follows: Grade 0=back-ground activity:1=faint uptake greater than background;2=definite uptake, but less than lumbar vertebrae; 3=same uptake as lumbar vertebrae, but less than liver; 4=same or higher uptake than liver. The results were as follows. 1) 42 of 65 (62%) patients with noninfectious nephritis showed grade 2 or higher Ga-67 renal uptake but only 10 percent of control patients showed similar uptake. 2) In 14 patients with systemic lupus erythematosus, 8 of 9 (89%) patients with lupus nephritis exhibited marked renal uptake 3) 36 of 41 patients (88%) with combined nephrotic syndrome showed Grade 2 or higher renal uptake. 4) Renal Ga-67 uptake was correlated with clinical severity of nephrotic syndrome determined by serum albumin level, 24 hr urine protein excretion and serum lipid levels. 5) After complete remission of nephrotic syndrome, renal uptake in all 8 patients who were initially Grade 3 or 4, decreased to Grade 1 or 0. In conclusion, we think that the mechanism of renal Ga-67 uptake in nephrotic syndrome might be related to the pathogenesis of nephrotic syndrome. In systemic lupus erythematosus, Ga-67 citrate scan is useful in predicting renal involvement.

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