• Childhood Kidney Diseases
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• v.24 no.2
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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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• v.20 no.2
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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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• v.25 no.1
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• pp.49-49
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• 2021

• Annals of Clinical Neurophysiology
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• v.9 no.1
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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.

• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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• v.17 no.2
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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.

• Journal of Veterinary Clinics
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• v.34 no.6
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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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• v.22 no.3
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• pp.210-215
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• 2015

Macrophage activation syndrome (MAS) is a rare complication in systemic lupus erythematosus (SLE) that can be triggered by infections. Due to the fact that MAS may mimic clinical features of underlying rheumatic disease, or be confused with an infectious complication, its detection can prove challenging. This is particularly true when there is an unknown/undiagnosed disease; and could turn into an even greater challenge if MAS and SLE are combined with a viral infection. A-14-year-old female came to the hospital with an ongoing fever for 2 weeks and a painful facial skin rash. Hepatomegaly, pancytopenia, increased aspartate aminotransferase, elevated serum ferritin and lactate dehydrogenase were reported. No hemophagocytic infiltration of bone marrow was reported. The patient was suspected for hemophagocytic lymphohistiocytosis. Her skin rashes were eczema herpeticum, which is usually associated with immune compromised conditions. With the history of oral ulcers and malar rash, positive ANA and low C3, C4 and the evidence of hemolytic anemia, she was diagnosed as SLE. According to the diagnostic guideline for MAS in SLE, she was diagnosed MAS as well, activated by acute HSV infection. After administering steroids and antiviral agent, the fever and skin rash disappeared, and the abnormal laboratory findings normalized. Therefore, we are reporting a rare case of MAS triggered by acute HSV infection as the first manifestation of SLE.

• Clinical and Experimental Pediatrics
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• v.53 no.2
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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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• v.31 no.4
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• pp.343-348
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• 2009

Kaposi's sarcoma was first descrided by Kaposi in 1872 as an idiopathic multiple hemorrhagic sarcoma. Its clinical features revealed to be erythematous red or purple macule started out, and developing into palpable dome-shaped nodules. Etiology is not defined to detail at present. Kaposi's sarcoma is classified to 4 categories; Classical, African, Epidemic and Immunosuppressive type. Epidemic categories is found approximately 20% of all AIDS patients and has strong predilection for head and neck region. The first case of immunosuppresive type Kaposi's sarcoma in patients with kidney transplants was reported in 1969. Kaposi's sarcoma accounts for 5% of all tumors associated with transplanted patients. The most common site of Kaposi's sarcoma in immunosuppressed patients are extremities, but rare in head and neck area. A 42 years old woman who had systemic lupus erythematosus visited to our clinic because of gingival hyperplasia, and excisional biopsy revealed Kaposi's sarcoma. We experienced a case of favorable results using excision and chemotherapy, so we report with review of literatures.

• The Journal of Korean Medicine
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• v.16 no.1 s.29
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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.