• Journal of Trauma and Injury
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• v.30 no.2
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• pp.59-62
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• 2017

Lupus thrombocytopenia is a common clinical manifestation in systemic lupus erythematosus (SLE). It may present to clinicians with considerable therapeutic difficulties. We experienced a 40-year-old poly trauma patient with lupus thrombocytopenia who had been treated with immunosuppressive drugs for SLE. She was treated for refractory thrombocytopenia with platelet transfusion, corticosteroid and Intravenous immunoglobulin (IVIG). Fourteen days after admission, her platelet count started to increase, $101{\times}103/ul$ at 16 days after admission. Trauma patients may carry various underlying diseases and thus trauma surgeons should always be aware and ready for peculiar situations.

• Clinical and Experimental Pediatrics
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• v.50 no.1
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• pp.74-78
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• 2007

Purpose : Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease with complex clinical manifestations. It probably involves genetic, environmental and immunologic factors. In this study, we investigated the clinical manifestations, laboratory findings and prognosis of pediatric SLE to aid clinical care of pediatric SLE. Methods : The data of 45 patients who were diagnosed as pediatric SLE in Severance Children's Hospital from Jan. 1996 to Dec. 2005 were analysed retrospectively. Results : The mean age at diagnosis was 10.8 (0-15) years old. And the ratio of male to female patients was 1:4. The initial manifestations were facial edema (51.1 percent), malar rash (44.4 percent), and fever (28.9 percent). The ANA (97.8 percent), anti-ds DNA antibody (82.2 percent), lupus nephritis (71.1 percent), malar rash (71.1 percent), and cytopenia (66.7 percent) were the most common findings among the classification criteria by ACR (American College of Rhematology, 1997). Conclusion : Clinical manifestations and prognosis are various in pediatric SLE. Intensive studies of SLE in children should be continued for more effective treatment.

• Archives of Plastic Surgery
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• v.38 no.3
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• pp.309-314
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• 2011

Purpose: Necrotizing fasciitis is a life-threatening, destructive soft tissue infection with a very high rate of mortality that needs early diagnosis and aggressive treatment. Systemic Lupus Erythematosus (SLE) is a systemic, autoimmune disease and it's major cause of mortality is an infection. But necrotizing fasciitis in SLE is very rare and there have been only 22 cases reported in the literatures. We reported a patient of necrotizing fasciitis with SLE and reviewed 22 others from literature research. Methods: A 40-year-old female patient with a history of SLE for 6 years came to the emergency room. The patient complained of severe pain and swelling on her right leg. She was diagnosed as necrotizing fasciitis and underwent emergency fasciotomy. As wound cultures showed variable organisms, she was treated with broad-spectrum antibiotics and underwent several surgical debridements. Then, the wound was treated with the V.A.C (Vacuum Assisted Closure) device and split thickness skin grafting was performed two times. Results: Skin graft was well taken within 2 weeks after operations and the patient was discharged to outpatient follow up. There was no complication related with surgery and she could walk without cane after 3 months. Conclusion: We treated a necrotizing fasciitis in a patient with SLE and reviewed 22 others from literature research. The case presented here suggests that necrotizing fasciitis is a rare disease in SLE patients, but should be considered in the differential diagnosis of soft tissue infection in SLE patients. A high index of suspicion is needed for early diagnosis and proper management in these patients.

• Childhood Kidney Diseases
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• v.28 no.2
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• pp.80-85
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• 2024

Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) is a rare entity characterized by the presence of lupus anticoagulant (LA) and prothrombin (factor II) deficiency. It may cause severe bleeding contrary to classical antiphospholipid syndrome. Here, we report a case of LAHPS presenting with a hemorrhagic ovarian cyst in a 17-year-old girl with systemic lupus erythematosus (SLE) nephritis. She had been followed up for 8 years. Her first manifestation of SLE was prolonged gingival bleeding after tooth extraction at 9 years of age. During the follow-up period, she had neither severe bleeding nor thrombotic complications despite a positive LA and a prolonged activated partial thromboplastin time (aPTT). At this visit, the patient presented with colicky abdominal pain, a hemorrhagic ovarian cyst, a prolonged prothrombin time, a prolonged aPTT, a low factor II level, and a positive LA, leading to the diagnosis of LAHPS. While a hemorrhagic ovarian cyst resolved completely in 3 months, she received oral pill, transfusions of red blood cells and plasma, and intravenous cyclophosphamide pulse therapy in combination with glucocorticoids due to persistent menorrhagia, anemia, prolonged aPTT, and lupus flaring. Thus, LAHPS needs to be considered in SLE patients with positive LA and prolonged aPTT.

• Clinical and Experimental Pediatrics
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• v.52 no.5
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• pp.611-614
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• 2009

Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease characterized by the production of a wide range of autoantibodies, resulting in tissue damage. Although the susceptibility to SLE has been attributed to complex interactions between genetic and environmental factors, the influence of a genetic predisposition to SLE is supported by observations of familial aggregations. Family studies have found that siblings with an SLE-affected relative have a 20-fold higher risk of developing SLE compared with the general population. Here, we present a rare case of two male siblings with SLE. The clinical, laboratory, and histopathological findings of these individuals showed the characteristic features of SLE. Human leukocyte antigen (HLA) typing revealed that the brothers and their mother shared the common HLA haplotype of DRB1*1501 and DQB1*0602, which is significantly associated with disease susceptibility in both family-based and casecontrol studies. This report provides an opportunity to reveal the role of genetic factors in the development of SLE.

• BMB Reports
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• v.39 no.2
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• pp.189-196
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• 2006

Alterations in the amino acid structure or sequence can generate neo-epitopes from self-proteins causing autoaggressive immune attack. Reactive nitrogen species are an important factor that induces post-translational modification of proteins by cellular reduction and oxidation mechanism; cysteinyl-nitrosylation or tyrosine nitration leading to potentially pathogenic pathways. It was thought of interest to investigate the immunogenicity of nitrated poly L-tyrosine vis-$\`{a}$-vis its possible role in the induction of antibodies in systemic lupus erythematosus (SLE). Commercially available poly L-tyrosine was exposed to nitrating species and the damage was monitored by UV spectroscopy and alkaline gel electrophoresis. The results indicated the formation of 3-nitrotyrosine. Nitrated poly L-tyrosine induced higher titre antibodies as compared to the native form. Nitrated poly L-tyrosine was recognized by the autoantibodies present in the sera of patients suffering from SLE by enzyme immunoassays and band shift assay. The possible role of nitrated self-proteins has been discussed in the production of circulating anti-DNA antibodies in SLE.

• The Journal of Internal Korean Medicine
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• v.44 no.2
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• pp.117-128
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• 2023

Objective: This study reported the improved symptoms of integrative Korean medicine-based treatment in a patient diagnosed with Systemic Lupus Erythematosus (SLE) who persistently complained of fatigue and chest tightness. Methods: A 51-year-old female SLE patient persistently complaining of fatigue and chest tightness underwent an 18-day treatment with Kracie Shihogayonggolmoryo-tang, Cheongpajeon-H, Gwanjeol-go, acupuncture, pharmacopuncture, cupping therapy, deep tissue meridian hot pack therapy, manual therapy, Interferential Current Therapy, and Extracorporeal Shock Wave Therapy. The Brief Fatigue Inventory (BFI) and Numeric Rating Scale (NRS) for each symptom were measured on admission, at one week, two weeks of hospitalization, and upon discharge. Clinical outcomes were assessed using these two figures. Results: After treatment, there was a reduction in the BFI score (7 to 3). The NRS score of chest tightness with hot flashes decreased from 8 to 5, neck pain lessened from 7 to 5, pain in both ankles diminished from 6 to 4, the cold hypersensitivity of both feet fell from 6 to 4, and heartburn decreased from 5 to 2. In order, the biggest clinical improvement was to heartburn, fatigue, and chest tightness with hot flashes. Conclusion: This case study shows that integrative Korean medicine-based treatment can improve symptoms of a Systemic Lupus Erythematosus patient persistently complaining of fatigue and chest tightness with hot flashes and suggests that Shihogayonggolmoryo-tang may be an effective option for managing and treating SLE patients.

• The Korean journal of internal medicine
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• v.39 no.2
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• pp.338-346
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• 2024

Background/Aims: Systemic lupus erythematosus (SLE) responder index (SRI)-4 response has been achieved with belimumab treatment in patients with moderate disease activity in cornerstone clinical trials and following studies. However, most studies involved patients treated with a mean prednisolone-equivalent dose of approximately 10 mg/d and focused on the steroid-sparing effect of belimumab. We aimed to identify the effect of belimumab in patients with mild-to-moderate SLE who were treated with low-dose or no corticosteroids. Methods: We retrospectively reviewed the electronic medical records of patients treated with belimumab for at least 6 months between May 2021 and June 2022. The primary endpoint was SRI-4 response at 6 months. Results: Thirty-one patients were included (13 low dose- and 18 steroid non-users). The mean age was 39.2 ± 11.4 years, and 90.3% of patients were female. The baseline Safety of Estrogens in Lupus Erythematosus National Assessment-Systemic Lupus Erythematosus Disease Activity Index (SELENA-SLEDAI) score was 6.0 (4.0-9.0). The primary endpoint was achieved in 32.3% (10/31) of patients. Significant improvements in anemia, C4 levels, and SELENA-SLEDAI score were observed during treatment. Univariate analysis showed that the baseline SELENA-SLEDAI and arthritis were significantly associated with SRI-4 response at 6 months, and only the SELENA-SLEDAI remained significant (p = 0.014) in multivariate analysis. Conclusions: This cohort study is the first to report the efficacy of belimumab after minimizing the effect of corticosteroids. Belimumab showed efficacy in improving the SELENA-SLEDAI score, anemia, and low C4 in patients who did not receive corticosteroids or received only low doses.

• Journal of Yeungnam Medical Science
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• v.22 no.2
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• pp.253-258
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• 2005

Generalized edema and hypoalbuminemia are relatively common presenting manifestations in many clinical situations. The differential diagnosis of hypoalbuminemia include: Kwashiorkor, synthetic dysfunction of the liver, and excessive protein loss as in nephrotic syndrome. In systemic lupus erythematosus (SLE), hypoalbuminemia and generalized edema are most commonly due to protein loss associated with lupus nephritis; gastrointestinal involvement is uncommon, and therefore protein loss through the gastrointestinal tract is quite rare. We report a case of a protein losing enteropathy (PLE) associated with SLE. The patient was referred to our hospital for generalized edema, arthralgia and facial rash. After clinical evaluation, the patient met the criteria for the SLE diagnosis; hypoalbuminemia with general edema was consistent with a protein losing enteropathy. After two weeks of therapy with parenteral high dose glucocorticoid, the patients was improved in laboratory findings as well as clinical symptoms.

• Journal of Haehwa Medicine
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• v.20 no.1
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• pp.11-23
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• 2011

Systemic Lupus Erythematosus(SLE) is an autoimmune disease invading the skin, joint, kidney, intestinal membrane, neurosystem and other organs. SLE is an autoimmune disease characterized by immune dysregulation resulting in the production of antinuclear antibodies(ANA), generation of circulating immune complexes, and activation of the complement system. In Korean medicine, lupus can be classified as acute arthritis, reddish butterfly erythema, asthenic disease, edema and so on. The cause and procedure of the diseases are flourishing noxious heat, excessive fire due to deficiency of yin, blood stasis due to stagnation of qi, internal movement of the liver-wind, congenital deficiency, exhausted vital-qi, which are treated by clearing away heat and cooling the blood, nourshing yin and extinguishing fire, treating flatulence and activating blood circulation, nourishing the blood to expel wind, invigorating the liver and kidney, invigorating qi and replenishing the blood. To experimentally examine the influence of Insam-Buja-Tang (Ginseng & Aconiti Extract, IBT) on the outbreak and development of lupus, lupus induce MRL/MpJ-Faslpr lupus-prone mice model was used. As IBT was orally administrated to a lupus model mouse, various tests such as the weight, urine protein, renal function, Lymph cell test of the spleen, Cytokine expression, histopathological analysis of kideny were performed to see the influence on the kidney and whether it work effectively on the immune function. The main purpose of this study is to evaluate the effect of IBT on MRL/MpJ-Faslpr lupus-prone mice model. The effect of IBT on MRL/MpJ-Faslpr lupus-prone mice that can have autoimmune disease similar to SLE in human was evaluated after IBT per oral in the present study.