• Childhood Kidney Diseases
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• 제24권2호
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• pp.107-114
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• 2020

Purpose: The purpose of this study was to evaluate the prevalence, clinical characteristics, and long-term clinical effects of hypertension in Korean childhood-onset systemic lupus erythematous (SLE) patients. Methods: The medical records of SLE patients, diagnosed by 2019 SLE European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria, who visited Samsung Medical Center from January 2009 to May 2019 were reviewed. Disease activity and long-term damage were evaluated using the Modified Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and the Pediatric Systemic Lupus International Collaborating Clinics/ACR Damage Index (Ped-SDI), respectively. The sex-, age- and height-blood pressure standards recommended by the American Academy of Pediatrics 2017 guideline was used to define hypertension. Results: A total of 32 patients were enrolled in this study. The median follow-up duration was 7.3 years and females were predominant. The median ages at SLE and hypertension diagnoses were 14.2 and 14.3 years, respectively. The biopsy-proven lupus nephritis was detected in 90.6% and 37.5% were class IV. During the follow-up, 12 patients (37.5%) had hypertension. Among them, 2 patients had 3 episodes of posterior reversible encephalopathy syndrome and 5 patients had left ventricular hypertrophy (LVH). Univariate analysis showed baseline hypertension was significantly correlated with a lower estimated glomerular filtration rate, higher body mass index and SLEDAI at baseline. The development of hypertension during the follow-up was significantly correlated with obesity, LVH, and higher Ped-SDI. Conclusion: Our study revealed that hypertension in pediatric SLE is associated with obesity and renal function at SLE diagnosis and could affect long-term damage.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제20권2호
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• pp.97-100
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• 1998

Systemic lupus erythematosus is a severe cutaneous-systemic disorder of unknown etiology, It is represented with erythematous patches on the face in a so-called butterfly distribution, and characteristically classified as an autoimmune disease with antinuclear antibodies. The autoimmune diseases such as systemic lupus erythematosus, $Sj{\ddot{o}}gren$ syndrome, rheumatoid arthritis have been associated with lymphoid malignancy - leukemia, malignant lymphoma - which could involve various organs(spleen, liver, brain, mediastinal lymph node, supraclavicular lymph node, inguinal lymph node, cervical lymph node etc.). Many authors have studied about the association of systemic lupus erythematosus and malignant lymphoma, but exact etiology is still unknown. A common viral etioloty for systemic lupus erythematosus has been suggested since virus-like particles have been found in the glomerular endothelium of patients with systemic lupus erythematosus. These oncogenic viruses may be responsible for the higher frequency of malignant lymphoma in patients with systemic lupus erythematosus. In the other theory, the causes of malignant lymphoma are the defect of immune system due to systemic lupus erythematosus and the long-term use of therapeutics for treatment of systemic lupus erythematosus. When the cellular immune system(delayed hypersensitivity) is impaired by immunosuppressive drugs, it is likely that the body is no longer able to recognize and reject malignant cells as they arise; they continue to grow and divide unhindered. The impairment of the cellular immune system may allow growth of oncogenic virus or the survival of neoplatic tissues. 47-year old female patient treated systemic lupus erythematosus with steroid and immunosuppressive drugs for 5 years visited to our hospital due to elevated mass on left upper anterior maxilla area. By performing biopsy, we diagnosed this lesion as malignant lymphoma and referred to oncologist for chemotherapy. So we report a case of malignant lymphoma due to systemic lupus erythematosus with review of literatures.

• Childhood Kidney Diseases
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• 제25권1호
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• pp.49-49
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• 2021

• Annals of Clinical Neurophysiology
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• 제9권1호
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• pp.29-32
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• 2007

Recurrent transverse myelitis is a rare manifestation of systemic lupus erythematosus. Recurrent transverse myelitis presents the biggest diagnostic problem, since it is common manifestation of multiple sclerosis. But it can also be the only feature or first manifestation in systemic lupus erythematosus. Neurological manifestations and magnetic resonance imaging can be indistinguishable, and there are no specific diagnostic tools. Here we describe a 59-year-old female having a systemic lupus erythematosus with recurrent transverse myeltitis. No uniform therapeutic protocol exists for systemic lupus erythematous with transverse myelitis, and the prognosis is usually poor. We suggest that aggressive treatment (usually with pulses of methylprednisolone and cyclophosphamide) might improve the prognosis of systemic lupus erythematosus with transverse myeltis.

• 한방안이비인후피부과학회지
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• 제17권2호
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• pp.140-145
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• 2004

Systemic lupus erythematosus is a disease of unknown etiology that affects many organ system and is characterized by the presence of multiple autoantibodies that participated in immunology mediated tissue injury. A 36 years-old female patient was admitted to ward due to high fever and erythematous rash on face and hole body. She exhibited itching sense, joint pain, nausea, fatigue, sensitivity to light. The homatologic finding revealed anemia, decrease of lymphocyte, low platelet count, but LE cell, Anti nuclear antibody(ANA) were negative. In the point of Differentiation of Syndrome(辨證), SLE can be thought to be a category of Seasonal febril disease(溫病). This subject diagnosed as Domination of intense evil heat(熱毒熾盛), and was administrated with Chungonpadocyem-gamibang(淸瘟敗毒飮加味方). The clinical and laboratory findings of our patient were improved by herb medication, acupuncture therapy and general supportive measure.

• 한국임상수의학회지
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• 제34권6호
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• pp.434-436
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• 2017

Nine dogs with history of lameness and anorexia were presented. On physical examination, all dogs had gait abnormality and six dogs had high body temperature. Their clinical signs were mostly episodic, and only non-specific symptoms were occasionally observed. Arthrocentesis was performed in all dogs, and immune-mediated polyarthritis (IMPA) was diagnosed. Definitive rheumatoid arthritis (RA) and systemic lupus erythematous (SLE) were diagnosed in one dogs, one each. Prednisolone (PDS) was chosen as the first-line therapy for all dogs, except for the one with RA. Most cases responded to PDS but some cases including those of SLE and RA were refractory to PDS. IMPA can be challenging to diagnose due to its vague symptom and is commonly implicated in 'fever of unknown origin'. Therefore, clinicians should consider IMPA as a differential diagnosis when the patient has fever with systemic, non-specific signs, such as anorexia and depression, but does not respond to antibiotics.

• Pediatric Infection and Vaccine
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• 제22권3호
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• pp.210-215
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• 2015

대식세포 활성 증후군(MAS, Macrophage activation syndrome)은 전신 홍반성 루푸스(SLE, systemic lupus erythematous) 환자에서 감염에 의해 나타날 수 있는 드문 합병증이다. MAS는 기저의 자가면역질환의 임상양상과 유사하게 나타나거나 혹은 감염성 합병증과 혼돈될 수 있어 감별에 주의 하여야 한다. 14세 여환이 2주간 지속되는 발열과 통증을 동반하는 얼굴의 피부 발진을 주소로 내원하였다. 피부 발진과 간비대, 범혈구 감소증, aspartate aminotransferase, lactate dehydrogenase, 혈청 ferritin이 상승하여, MAS를 의심하였다. 피부 병변과 항핵체 양성, C3와 C4의 감소, 간접 쿰스검사 양성으로 SLE를 진단하였다. 따라서 본 증례는 MAS가 HSV에 의하여 촉발된 것을, SLE의 첫 증상으로서 나타낸 증례로서, 촉발 요인 및 기저질환을 치료함으로써 중증의 합병증 없이 호전되었다.

• 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.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제31권4호
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• pp.343-348
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• 2009

본 증례는 전신성 홍반성 낭창으로 인해 2000년 4월부터 면역 억제제인 azathioprine 75mg과 prednisolne 35mg을 복용 중인 42세 여성 환자가 2004년 12월 상악 협측 치은의 종창, 증식, 동통 및 출혈을 주소로 내원하여 조직 생검 결과 모세 혈관종으로 진단되었으나, 2005년 1월 치은의 재발 병소의 절제 및 조직 생검으로 카포시 육종으로 진단되었고, 흉부의 피부 병변의 조직 생검과 흉부 전산화 단층 촬영 검사에서도 양측 폐에 다수의 결절을 보이는 카포시 육종으로 진단되었다. 의인성 면역억제제에 의한 카포시 육종으로 최종 진단되어, 면역 억제제와 부신피질호르몬제의 중단과, paclitaxel을 이용한 전신적인 항암화학 요법으로, 2008년11월 현재까지 재발의 징후 없이 구강, 피부, 폐의 병소에 대해 양호한 치료 결과를 얻었으나, 향후 전신성 홍반성 낭창의 악화 또는 카포시 육종의 재발 가능성이 있으므로 지속적인 추적 관찰이 요할 것으로 사료된다.

• 대한한의학회지
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• 제16권1호통권29호
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• pp.51-70
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• 1995

To find the cause, pathology, oriental presciptions for SLE(systemic lupus erythematous) in the field of oriental medicine, we studied the clinical reports. The results were obtained as follows: 1. SLE can' be thought to be a category of chronic fatigue, numbness, yang poison' & erythema. 2. The chief cause of SLE could be a febrile poison and the lack of physiological fluid and the mechanism was that febrile poison attacted area of circulation and blood and injuried the physiological fluid to arise edema and clotted blood. 3. The therapy of SLE was chiefly clarifying heat and toxiciding in acute stage while reinforcing kidney and liver and supplementing Yin & clarifying in chronic stage, sometimes supplementing Qi, eliminating clotted blood, excuding wind and draining water could be applied. 4. The therapy for SLE nephritis was shown to be increasing yin and clarifying heat with toxiciding, reinforcing spleen and kidney, draining water, supplementing yin & Qi etc. The chief prescriptions were Seogakjihwangtang(犀角地黃湯), Wookakjihwangtang(牛角地黃湯), yeechihwan(二至丸), Daepowonjeon(大補元煎), Daepoeumhwan(大補陰煎), Kikukjihwangtang(杞菊地黃湯), Yookmijihwangtang(六味地黃湯) which indicates Rhemanniae radix added prescrition could be used chiefly for the treatment of SLE.