• Title/Summary/Keyword: Systemic Lupus Erythematosus

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Corticosteroid-induced Avascular Necrosis of Talus after Cardiac Transplantation (심장이식 후 부신피질호르몬 투여로 인한 거골의 무혈성 괴사 - 1예 보고 -)

  • Park, Hong-Gi;Eom, Gi-Serk
    • Journal of Korean Foot and Ankle Society
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    • v.4 no.2
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    • pp.83-86
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    • 2000
  • A vascular necrosis of the talus has frequently been reported following trauma because talus has no muscle insertions, sixty percent of the surface of the talus is covered by hyaline cartilage, takes only a small area for entrance of a blood supply. Osteonecrosis is also associated with a variety of nontraumatic disorders. There are many indications for steroid usage, patient with rheumatoid arthritis, systemic lupus erythematosus, chronic obstructive pulmonary disease, and status- post renal or cardiac transplantation may be on long- term steroid usage, osteonecrosis may develop. A vascular necrosis of the talus secondary to chronic steroid usage is an unusual case. Delay in detection of osteonecrosis may lead to fragmentation and collapse of the talar body. When pain on range of motion is present and conservative treatment have been exhausted, surgical treatment is indicated, that is, fusion of the ankle joint. However it is important that conservative treatment may prevent its various sequelae with early diagnosis because steroid - treated patients have a more operative risk and increased risk for postoperative infection. We report a rare case of corticosteroid induced avascular necrosis of talus after cardiac transplantation.

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Two Cases of C1q Nephropathy in Siblings (남매에서 발생한 C1q 신증 2례)

  • Kim, Su-Young;Kim, Seong-Heon;Moon, Kyung-Chul;Shin, Jae-Il;Jeong, Hyen-Joo
    • Childhood Kidney Diseases
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    • v.16 no.1
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    • pp.46-50
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    • 2012
  • C1q nephropathy is a distinct clinicopathologic entity, characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence for systemic lupus erythematosus. Clinically it may present as nephrotic syndrome and non-nephrotic proteinuria per se or associated with microscopic hematuria, gross hematuria, hypertension, or renal insufficiency. So far there is only one report about a familial case of C1q nephropathy (in two sisters). We present two cases of familial C1q nephropathy with nephrotic syndrome which was steroid resistant, but partially remitted with cyclosporine.

The Role of Thymic Stromal Lymphopoietin (TSLP) in Glomerulonephritis

  • Lee, Keum Hwa;Yang, Jae Won;Cho, Jin Young;Lee, Joo Yup;Lim, Eun Kyung;Eisenhut, Michael;Jeong, Dong Yeon;Steingroever, Johanna;Shin, Jae Il
    • Childhood Kidney Diseases
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    • v.22 no.1
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    • pp.17-21
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    • 2018
  • Thymic stromal lymphopoietin (TSLP) is an interleukin-7-like cytokine that is an important trigger and initiator of many allergic diseases. TSLP promotes a T-helper type 2 (Th2) cytokine response that can be pathological. A relationship is formed both at the induction phase of the Th2 response through polarization of dendritic cells to drive Th2 cell differentiation and at the effector phase of the response, by promoting the expansion of activated T cells and their secretion of Th2 cytokines and TSLP. In transgenic mice with TSLP overexpression, it has been reported that TSLP leads to the development of mixed cryoglobulinemic membranoproliferative glomerulonephritis. In addition, TSLP can play an important role in the pathogenesis of IgA nephropathy and systemic lupus erythematosus-related nephritis. From our knowledge of the role of TSLP in the kidney, further studies including the discovery of new therapies need to be considered based on the relationship between TSLP and glomerulonephritis.

Myocardial Revascularization in Two Patients Associated with Antiphospholipid Syndrome: Different Pathogenic Patterns and Angiographic Results

  • Park, Samina;Hwang, Ho-Young;Kang, Hyun-Jae;Kim, Ki-Bong
    • Journal of Chest Surgery
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    • v.44 no.6
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    • pp.423-426
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    • 2011
  • We report on two women who underwent myocardial revascularization associated with antiphospholipid syndrome (APS) with different pathogenic patterns. The first woman presented with acute myocardial infarction, and preoperative angiograms demonstrated rapidly progressing coronary lesions, presumptive unstable plaque, and dissection. Operative findings, however, showed fresh thrombi in the coronary arteries, and she was diagnosed postoperatively as having APS. Her one-year angiogram demonstrated improved coronary lesions and a competitive flow pattern in the grafts. The second woman presented with unstable angina and had been treated for systemic lupus erythematosus and secondary APS for more than 14 years. She underwent myocardial revascularization due to accelerated coronary atherosclerosis. Her one-year angiogram demonstrated patent grafts.

Preclinical Efficacy and Mechanisms of Mesenchymal Stem Cells in Animal Models of Autoimmune Diseases

  • Lee, Hong Kyung;Lim, Sang Hee;Chung, In Sung;Park, Yunsoo;Park, Mi Jeong;Kim, Ju Young;Kim, Yong Guk;Hong, Jin Tae;Kim, Youngsoo;Han, Sang-Bae
    • IMMUNE NETWORK
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    • v.14 no.2
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    • pp.81-88
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    • 2014
  • Mesenchymal stem cells (MSCs) are present in diverse tissues and organs, including bone marrow, umbilical cord, adipose tissue, and placenta. MSCs can expand easily in vitro and have regenerative stem cell properties and potent immunoregulatory activity. They inhibit the functions of dendritic cells, B cells, and T cells, but enhance those of regulatory T cells by producing immunoregulatory molecules such as transforming growth factor-${\beta}$, hepatic growth factors, prostaglandin $E_2$, interleukin-10, indolamine 2,3-dioxygenase, nitric oxide, heme oxygenase-1, and human leukocyte antigen-G. These properties make MSCs promising therapeutic candidates for the treatment of autoimmune diseases. Here, we review the preclinical studies of MSCs in animal models for systemic lupus erythematosus, rheumatoid arthritis, Crohn's disease, and experimental autoimmune encephalomyelitis, and summarize the underlying immunoregulatory mechanisms.

Methotrexate-induced Oral Mucositis

  • Lee, Hye-Jin;Kwon, Jeong-Seung;Choi, Young-Chan;Ahn, Hyung Joon
    • Journal of Oral Medicine and Pain
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    • v.40 no.2
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    • pp.82-87
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    • 2015
  • Methotrexate (MTX) is a chemotherapeutic agent that is used to treat a host of malignancies. But recently, MTX has also been used as a therapeutic agent for chronic inflammatory disorders such as rheumatoid arthritis, psoriasis, and systemic lupus erythematosus. However, MTX is an antimetabolite that affects rapidly dividing normal cells such as oral mucosal epithelial cells, gastrointestinal epithelial cells, and bone marrow cells-which explains why oral mucositis is often an initial manifestation of MTX toxicity. Because oral lesions are frequently initially presented in dental clinics, dentists should consider the possibility of adverse drug reactions in the differential diagnoses of oral lesions through a meticulous collection of patients' medical histories. In this report, we examine patients who suffered from oral ulcerative lesions upon diagnosis of MTX-induced oral mucositis. Then, we suggest approaches for the diagnosis and treatment of MTX-induced oral mucositis through a review of literature.

A Case of Chylothorax after Tube Thoracostomy (흉강삽관술 후 발생한 유미흉 1예)

  • Choi, Kyu-Un;Kang, Gyung-Hoon;Kim, Sung-Hoon;Seo, Hyun-Woong;Jung, Bock-Hyun;Kim, Sung-Soo;Lim, Jae-Min
    • Tuberculosis and Respiratory Diseases
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    • v.72 no.1
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    • pp.59-62
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    • 2012
  • Tube thoracostomy is known to cause complications such as bleeding or infection, but the incidence of chylothorax secondary to tube thoracostomy is under-reported, and therefore, we report this case. A patient was diagnosed as systemic lupus erythematosus with pleural and pericardial involvement. During repeated therapeutic thoracentesis, which were performed because of poor response to steroids and cylophosphamide, hemothorax developed and we therefore inserted a chest tube. The pleural effusion changed from red to milky color in several hours and we diagnosed the pleural effusion as chylothorax. Total parenteral nutrition based on medium-chain triglycerides was supplied to this patient and chylothorax was improved after 4 days.

Targeted Immunotherapy for Autoimmune Disease

  • Seung Min Jung;Wan-Uk Kim
    • IMMUNE NETWORK
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    • v.22 no.1
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    • pp.9.1-9.23
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    • 2022
  • In the past few decades, biological drugs and small molecule inhibitors targeting inflammatory cytokines, immune cells, and intracellular kinases have become the standard-of-care to treat autoimmune diseases. Inhibition of TNF, IL-6, IL-17, and IL-23 has revolutionized the treatment of autoimmune diseases, such as rheumatoid arthritis, ankylosing spondylitis, and psoriasis. B cell depletion therapy using anti-CD20 mAbs has shown promising results in patients with neuroinflammatory diseases, and inhibition of B cell survival factors is approved for treatment of systemic lupus erythematosus. Targeting co-stimulatory molecules expressed on Ag-presenting cells and T cells is also expected to have therapeutic potential in autoimmune diseases by modulating T cell function. Recently, small molecule kinase inhibitors targeting the JAK family, which is responsible for signal transduction from multiple receptors, have garnered great interest in the field of autoimmune and hematologic diseases. However, there are still unmet medical needs in terms of therapeutic efficacy and safety profiles. Emerging therapies aim to induce immune tolerance without compromising immune function, using advanced molecular engineering techniques.

The Role of Plasmacytoid Dendritic Cells in Gut Health

  • Hye-Yeon Won;Ju-Young Lee;Dahye Ryu;Hyung-Taek Kim;Sun-Young Chang
    • IMMUNE NETWORK
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    • v.19 no.1
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    • pp.6.1-6.14
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    • 2019
  • Plasmacytoid dendritic cells (pDCs) are a unique subset of cells with different functional characteristics compared to classical dendritic cells. The pDCs are critical for the production of type I IFN in response to microbial and self-nucleic acids. They have an important role for host defense against viral pathogen infections. In addition, pDCs have been well studied as a critical player for breaking tolerance to self-nucleic acids that induce autoimmune disorders such as systemic lupus erythematosus. However, pDCs have an immunoregulatory role in inducing the immune tolerance by generating Tregs and various regulatory mechanisms in mucosal tissues. Here, we summarize the recent studies of pDCs that focused on the functional characteristics of gut pDCs, including interactions with other immune cells in the gut. Furthermore, the dynamic role of gut pDCs will be investigated with respect to disease status including gut infection, inflammatory bowel disease, and cancers.

Predictors of renal relapse in Koreans with lupus nephritis after achieving complete response: a 35-years of experience at a single center

  • Howook Jeon;Jennifer Lee;Su-Jin Moon;Seung-Ki Kwok;Ji Hyeon Ju;Wan-Uk Kim;Sung-Hwan Park
    • The Korean journal of internal medicine
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    • v.39 no.2
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    • pp.347-359
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    • 2024
  • Background/Aims: Renal relapse has known to be a poor prognostic factor in patients with lupus nephritis (LN), but there were few studies that identified the risk factors of renal relapse in real world. We conducted this study based on 35-years of experience at a single center to find out predictors of renal relapse in Korean patients with LN after achieving complete response (CR). Methods: We retrospectively analyzed the clinical, laboratory, pathologic and therapeutic parameters in 296 patients of LN who reached CR. The cumulative risk and the independent risk factors for renal relapse were examined by Kaplan-Meier methods and Cox proportional hazards regression analyses, respectively. Results: The median follow-up period from CR was 123 months. Renal relapse had occurred in 157 patients. Renal relapse occurred in 38.2%, 57.6% and 67.9% of patients within 5-, 10-, and 20-year, respectively. The age at diagnosis of SLE and LN were significantly younger, and the proportions of severe proteinuria and serum hypoalbuminemia were higher in patients with renal relapse. Interestingly, the proportion of receiving cytotoxic maintenance treatment was higher in patients with renal relapse. In Cox proportional hazards regression analyses, only young-age onset of LN (by 10 years, HR = 0.779, p = 0.007) was identified to independent predictor of renal relapse. Conclusions: Young-age onset of LN was only independent predictor and the patients with severe proteinuria and serum hypoalbuminemia also tended to relapse more, despite of sufficient maintenance treatment. Studies on more effective maintenance treatment regimens and duration are needed to reduce renal relapse.