• Childhood Kidney Diseases
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• 제25권1호
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• pp.40-43
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• 2021

Enuresis is intermittent urinary incontinence during sleep at night in children aged 5 years or older. The main pathophysiology of enuresis involves nocturnal polyuria, abnormal sleep arousal, and low functional bladder capacity. In rare cases, enuresis is an early symptom of endocrine disorders such as diabetes or thyroid disorders. Herein, we report a case of a 12-year-old girl with enuresis as a rare initial presentation of Graves' disease. She complained of nocturnal enuresis from a month before visiting our clinic. She also complained of urinary frequency, headache, and weight loss. On physical examination, she had tachycardia, intention tremors, and a diffuse goiter on her anterior neck with bruit on auscultation. Her thyroid function test results revealed hyperthyroidism, and Graves' disease was diagnosed as the thyroid stimulating hormone receptor autoantibody was positive. After treatment for Graves' disease with methimazole, symptoms of enuresis resolved within 2 weeks as she became clinically and biochemically euthyroid. In children with secondary enuresis, Graves' disease should be considered as a differential diagnosis, and signs of hyperthyroidism should be checked for carefully.

• IMMUNE NETWORK
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• 제22권1호
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• pp.7.1-7.20
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• 2022

Recently, there have been impressive advancements in understanding of the immune mechanisms underlying cutaneous inflammatory diseases. To understand these diseases on a deeper level and clarify the therapeutic targets more precisely, numerous studies including in vitro experiments, animal models, and clinical trials have been conducted. This has resulted in a paradigm shift from non-specific suppression of the immune system to selective, targeted immunotherapies. These approaches target the molecular pathways and cytokines responsible for generating inflammatory conditions and reinforcing feedback mechanisms to aggravate inflammation. Among the numerous types of skin inflammation, psoriasis and atopic dermatitis (AD) are common chronic cutaneous inflammatory diseases. Psoriasis is a IL-17-mediated disease driven by IL-23, while AD is predominantly mediated by Th2 immunity. Autoimmune bullous diseases are autoantibody-mediated blistering disorders, including pemphigus and bullous pemphigoid. Alopecia areata is an organ-specific autoimmune disease mediated by CD8⁺ T-cells that targets hair follicles. This review will give an updated, comprehensive summary of the pathophysiology and immune mechanisms of inflammatory skin diseases. Moreover, the therapeutic potential of current and upcoming immunotherapies will be discussed.

• Tuberculosis and Respiratory Diseases
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• 제72권2호
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• pp.197-202
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• 2012

Microscopic polyangiitis is a necrotizing vasculitis, characterized by inflammation of small vessels (capillaries, venules, and arterioles) with few or no immune deposits. The kidneys are the most commonly affected organs and are involved in 90% of patients, whereas pulmonary involvement occurs in a minority of cases (10% to 30%). In cases of lung disease, diffuse alveolar hemorrhage with pulmonary capillaritis is the most common manifestation. Microscopic polyangiitis is strongly associated with antineutrophil cytoplasmic autoantibody, which is a useful diagnostic serological marker. We report a case of microscopic polyangiitis presented as pleural effusion in a 67-year-old female. Pleural effusions have been reported in some cases previously, but the number of cases were small and their characteristics have not been well described. This report describes characteristic findings of pleural fluid and its histological features in a case of microscopic polyangiitis.

• Molecules and Cells
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• 제25권1호
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• pp.64-69
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• 2008

Although the arthritis symptoms observed in the K/BxN model have been shown to be dependent on the functions of T and B cells specific to the self antigen glucose-6-phosphate isomerase, less is known about the in vivo roles of $CD4^{+}CD25^{+}$ regulatory T($T_{reg}$) cells in the pathology of K/BxN mice. We determined the quantitative and functional characteristics of the $T_{reg}$ cells in K/BxN mice. These mice contained a higher percentage of $Foxp3^+\;T_{reg}$ cells among the $CD4^+$ T cells than their BxN littermates. These $T_{reg}$ cells were anergic and efficiently suppressed the proliferation of $na\ddot{i}ve$ $CD4^+$ T cells and cytokine production by effector $CD4^+$ T cells in vitro. Antibody-mediated depletion of $CD25^+$ cells caused K/BxN mice to develop multi-organ inflammation and autoantibody production, while the symptoms of arthritis were not affected. These results demonstrate that despite the inability of the $T_{reg}$ cells to suppress arthritis development, they play a critical role protecting the arthritic mice from systemic expansion of autoimmunity.

• Tuberculosis and Respiratory Diseases
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• 제69권5호
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• pp.375-380
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• 2010

Lymphoid interstitial pneumonia (LIP) is a rare benign lymphoproliferative interstitial lung disease. LIP has been associated with autoimmune disorders, HIV, viral infections, and so on. Once underlying systemic diseases have been excluded, a diagnosis of idiopathic LIP can be made. Although 6 cases of pathologically confirmed LIP have occurred in Korea, thus far none has been associated with primary Sjogren's syndrome. A 44-year-old man was admitted to hospital due to a dry cough and dypsnea on exertion that had been ongoing for 2 months. A chest radiography showed multiple and variable-sized cystic lesions, on both lungs and both interstitial infiltration and consolidation in both lower lung fields. Tests for autoantibody showed positive results of anti-nuclear antibody and anti-Ro/La antibody. The patient underwent a video assisted thoracoscopic surgery biopsy and pathologically confirmed LIP. We report the first known case of LIP-associated with primary Sjogren's syndrome in Korea.

• 한국임상약학회지
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• 제25권3호
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• pp.166-170
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• 2015

Background: Primary warm autoimmune hemolytic anemia (AIHA) is a relatively rare hematologic disorder resulting from autoantibody production against red blood cells. There has been very few studies about primary warm AIHA in South Korea because of its low incidence. We retrospectively analyzed the treatment outcome of primary warm AIHA. Method: We reviewed retrospectively the medical records of 9 primary warm AIHA patients from December 2002 to January 2015. We analyzed the causes and clinical characteristics of primary warm AIHA patients. We retrospectively analyzed the clinical data in electronic medical records for 9 Korean patients with AIHA patients who were diagnosed during the period from December 2002 to January 2015 at the Regional University Hospital in Korea. The study protocol was approved by the Institutional Review Board (IRB #2015-08-007, Chosun University Hospital IRB). Results: The mean age was 52 years (range 27~78), the mean hemoglobin level was 5.0 g/dL (range 2.5~6.4 g/dL). All patients received steroids at therapeutic dosages (corticosteroid 1 mg/Kg) as first line treatment. Eight of them showed complete response (5/8, 62.5%) and partial response (3/8, 37.5%), one patient required second-line treatment with rituximab. Two patients who responded first line treatment were relapsed at 86 weeks and 24 weeks after response, respectively. Only one patient of them was retreated with corticosteroid because of anemic symptoms. Conclusion: This study indicates that oral corticosteroid is an effective therapy for primary warm AIHA.

• Journal of Yeungnam Medical Science
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• 제21권1호
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• pp.101-107
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• 2004

Diffuse alveolar hemorrhage is a rare but serious and frequently life-threatening complication of a variety of conditions. The first goal in the management of patients with diffuse alveolar hemorrhage is to achieve or preserve stability of the respiratory status. Subsequently, the differential diagnosis is aimed at the identification of a remediable cause of the alveolar hemorrhage. The most common causes of diffuse alveolar hemorrhage with glomerulonephritis are microscopic polyangiitis and Wegener's granulomatosis, followed by Goodpasture syndrome and systemic lupus erythematosus. Microscopic polyangiitis (MPA) is a distinct systemic small vessle vasculitis affecting small sized vessels with few or no immune deposits and with no granulomatosus inflammation. The disease may involve multiple organs such as kidney, lung, skin, joint, muscle, gastrointestinal tract, eye, and nervous system. MPA is strongly associated with antineutrophil cytoplasmic autoantibody (ANCA) that is a useful serological diagnostic marker for the most common form of necrotizing vasculitis. Our report concerns a case of microscopic polyangiitis with diffuse alveolar hemorrhage in a 54-year-old man. He was admitted to our hospital due to dyspnea upon exertion and recurrent hemoptysis. Laboratory findings showed hematuria, proteinuria and deterioration of renal function. In the chest CT scan, diffuse ground glass appearance was seen in both lower lungs. A lung biopsy revealed small vessel vasculitis with intraalveolar hemorrhage and showed a positive reaction to against perinuclear ANCA. The patient was treated with prednisolone and cyclophosphamide. Chest infiltration decreased and hemoptysis and hypoxia improved. He is still being followed up in our hospital with a low dose of prednisolone.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제31권5호
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• pp.414-418
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• 2009

Pemphigus vulgaris is a chronic autoimmune intraepithelial blistering disease with oral mucosal manifestations that very often precede the skin lesions. The vesicles or bullae are produced by an acantholytic process, detachment of differentiating keratinocytes from one another in the epithelial stratum spino sum or spinous cell layer. The pathogenesis of this disease is initially manifested by IgG(mainly) binding to desmosome(desmoglein 3 or 1) in the intercellular spaces of epithelium. This autoantibody binding caused the release of a plasminogen activator(a proteolytic enzyme) from keratinocytes. This ultimately results in cell to cell separation. The mainstay therapy of pemphigus vulgaris is systemic corticosteroids and immunosuppressive agents to eliminate the pathogenic autoantibodies from circulation. A 41-year old woman presented with a 1.5 year history of oral ulceration. There were no lesions on the skin or other mucosal sites. Histology and immunostaining were consistent with pemphigus vulgaris. Control of oral ulceration and normal oral function were achieved after systemic corticosteroids and immunosuppressive agents were instituted.

• Clinical and Experimental Reproductive Medicine
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• 제31권3호
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• pp.149-154
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• 2004

Objective: To assess the association with autoimmune endocrine diseases and detection rate of autoimmune antibodies and its clinical significance in patients with premature ovarian failure. Methods: Twenty eight patients with primary or secondary amenorrhea manifesting hormonal and clinical features of premature ovarian failure (primary POF: 7, secondary POF: 21) were investigated. We tested them TFT, 75 g OGTT, ACTH and S-cortisol for thyroiditis, IDDM, Addison's disease, and antithyoglobulin antibody, antimicrosomal antibody, antinuclear antibody, rheumatic factor, anti-smooth muscle antibody, anti-acetylcholine receptor antibody for non-organ specific autoimmune disorders. Results: Only one patient was diagnosed as IDDM and no patients had abnormal TFT or adrenal function test. More than one kind of autoantibody was detected in 11 patients of all (39.2%): 5 patients (71.4%) of primary POF group and 6 patients (21.4%) of secondary POF group. Eleven patients (39.3%) had antithyroglobulin antibody, 4 (14.3%) had antimicrosomal antibody, 2 (7.1%) had antinuclear antibody, 2 (7.1%) had rheumatic factor, 1 (3.6%) had anti-smooth muscle antibody, 1 (3.6%) had anti-acetylcholine receptor antibody. Conclusions: Premature ovarian failure may occur as a component of an autoimmune polyglandular syndrome, so patients should be measured with free thyroxine, thyroid-stimulating hormone, fasting glucose and electrolytes. Measurement of thyroid autoantibodies in POF patients may be important in identifying patients at risk of developing overt hypothyoidism, but other autoantibodies may not be suitable for screening test.

• Biomolecules & Therapeutics
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• 제25권2호
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• pp.130-139
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• 2017

$CXCR5^+$ T follicular helper (Tfh) cells are associated with aberrant autoantibody production in patients with antibody-mediated autoimmune diseases including lupus. Follicular regulatory T (Tfr) cells expressing CXCR5 and Bcl6 have been recently identified as a specialized subset of $Foxp3^+$ regulatory T (Treg) cells that control germinal center reactions. In this study, we show that retroviral transduction of CXCR5 gene in $Foxp3^+$ Treg cells induced a stable expression of functional CXCR5 on their surface. The Cxcr5-transduced Treg cells maintained the expression of Treg cell signature genes and the suppressive activity. The expression of CXCR5 as well as Foxp3 in the transduced Treg cells appeared to be stable in vivo in an adoptive transfer experiment. Moreover, Cxcr5-transduced Treg cells preferentially migrated toward the CXCL13 gradient, leading to an effective suppression of antibody production from B cells stimulated with Tfh cells. Therefore, our results demonstrate that enforced expression of CXCR5 onto Treg cells efficiently induces Tfr cell-like properties, which might be a promising cellular therapeutic approach for the treatment of antibody-mediated autoimmune diseases.