• 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.

• 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.17 no.2
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• pp.98-103
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• 2014

Purpose: Gallbladder (GB) wall thickening can be found in various conditions unrelated to intrinsic GB disease. We investigated the predisposing etiologies and the outcome of acalculous GB wall thickening in children. Methods: We retrospectively analyzed 67 children with acalculous GB wall thickening who had visited our institute from June 2010 to June 2013. GB wall thickening was defined as a GB wall diameter > 3.5 mm on abdominal ultrasound examination or computed tomography. Underlying diseases associated with GB wall thickening, treatment, and outcomes were studied. Results: There were 36 boys and 31 girls (mean age, $8.5{\pm}4.8years$ [range, 7 months-16 years]). Systemic infection in 24 patients (35.8%), acute hepatitis in 18 (26.9%), systemic disease in 11 (16.4%), hemophagocytic lymphohistiocytosis in 4 (6.0%), acute pancreatitis in 3 (4.5%), and specific liver disease in 3 (4.5%) predisposed patients to GB wall thickening. Systemic infections were caused by bacteria in 10 patients (41.7%), viruses in 5 patients (20.8%), and fungi in 2 patients (8.3%). Systemic diseases observed were systemic lupus erythematosus in 2, drug-induced hypersensitivity in 2, congestive heart failure in 2, renal disorder in 2. Sixty-one patients (91.0%) received symptomatic treatments or treatment for underlying diseases. Five patients (7.5%) died from underlying diseases. Cholecystectomy was performed in 3 patients during treatment of the underlying disease. Conclusion: A wide range of extracholecystic conditions cause diffuse GB wall thickening that resolves spontaneously or with treatment of underlying diseases. Surgical treatments should be avoided if there are no definite clinical manifestations of cholecystitis.

• IMMUNE NETWORK
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• v.4 no.3
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• pp.161-165
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• 2004

Background: Anti-cardiolipin antibody (Anti-CL Ab) is one of the various antiphospholipid antibodies (Anti-PL Abs) and found in the plasma of patients with systemic lupus erythematosus (SLE), atherosclerosis, and other infectious diseases. While anti-PL Abs found in the sera of patients with infectious diseases bind directly to CL, binding of anti-PL Abs to CL circulating in the sera of patients with autoimmune diseases is mediated by $\beta_2-$glycoprotein 1 ($\beta_2-GP1$). The purpose of this study is to investigate the effect of <$\beta_2-GP1$ on the antigen binding assay of anti-CL Abs present in the sera of patients with atherosclerosis, which has been known as one of autoimmune diseases. Methods: ELISA was performed with sera containing anti-CL Abs from three patients with atherosclerosis in the presence or absence of $\beta_2-GP1$ or FBS. Results: Reactivity of anti-CL Abs to CL was increased in the presence of $\beta_2-GP1$ or FBS in a dose dependent manner. Conclusion: <$\beta_2-GP1$ or FBS could be used as co-factor in CL ELISA with anti-CL Abs present in the sera of patients with atherosclerosis. It is suggested that anti-CL Abs found in atherosclerosis patients are similar in terms of antigen binding property to those circulating in the patients with autoimmune diseases, not to infectious diseases.

• Korean Journal of Head & Neck Oncology
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• v.16 no.2
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• pp.212-215
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• 2000

Background and Objective: Kikuchi's disease(KD) is an idiopathic, self-limited lymphadenopathy that was described as a distinctive type of necrotizing lymphadenitis affecting primarily cervical lymph nodes of young adults independently by Kikuchi and Fujimoto et al at first in 1972. The purpose of this study is a knowledge about clinicopathologic findings, many laboratory tests and differentiation of KD from other lymphadenitis due to lymphoma, systemic lupus erythematosus(SLE) and many viral disease. Materials and Methods: Thirty-four case of KD collected at Chonnam University Hospital in Kwang-Ju from 1992 through 2000 were evaluated with retrospective chart review. Results: The patients were consisted of 11 men and 23 women. All patients had tender or nontender cervical mass and fever was the most common associated symptom. The others was pain, weight loss, chills, cold sweating and headache et al. Multiple bilateral involvement of cervical lymphnodes was 25 cases(74%) and solitary involvement was 9 cases(26%). In laboratory tests, leukopenia was 12 cases(75%), elevated ESR 5 cases (34%) and elevated LDH 11 cases(69%). Conclusion: KD is necessary to differentiate from lymphoma and SLE, because of the different of therapeutic modality and prognosis. The diagnosis is established on the basis of histopathologic studies with excisional biopsy of lymph node.

• Proceedings of the Korean Society of Veterinary Clinics Conference
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• 2006.05a
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• pp.113-113
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• 2006

• Clinical and Experimental Pediatrics
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• v.59 no.6
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• pp.252-255
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• 2016

Purpose: Pneumocystis jirovecii pneumonia occurs in various immunocompromised patients. Despite the prophylaxis strategies in clinical practice, certain patients develop P. jirovecii pneumonia. This study was performed to investigate pediatric cases with P. jirovecii pneumonia in a single center. Methods: We identified pediatric patients younger than 19 years with microbiologically confirmed P. jirovecii pneumonia from January 2000 to February 2014. A retrospective chart review was performed. Results: Fifteen episodes of P. jirovecii pneumonia in 14 patients were identified with median age of 8.3 years (range, 0.4-18.6 years). Among these patients, 11 patients had hematology-oncology diseases, 2 had primary immunodeficiency disorders (one with severe combined immunodeficiency and the other with Wiskott Aldrich syndrome), 1 had systemic lupus erythematosus and 1 received kidney transplant. Four patients were transplant recipients; 1 allogeneic and 2 autologous hematopoietic cell transplant and 1 with kidney transplant. The median absolute lymphocyte count at the diagnosis of P. jirovecii pneumonia was $5,156cells/mm^3$ (range, $20-5,111cells/mm^3$). In 13 episodes (13 of 15, 86.7%), patients were not receiving prophylaxis at the onset of P. jirovecii pneumonia. For treatment, trimethoprim/sulfamethoxazole was given as a main therapeutic agent in all 15 episodes. Steroid was given in 9 episodes (60%). Median treatment duration was 15 days (range, 4-33 days). Overall mortality at 60 days was 35.7% (5 of 14). Conclusion: Majority of our patients developed P. jirovecii pneumonia while not on prophylaxis. Continuous efforts and more data are needed to identify high risk patients who may get benefit from P. jirovecii pneumonia prophylaxis.

• Journal of Yeungnam Medical Science
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• v.21 no.1
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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.

• Clinical and Experimental Pediatrics
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• v.51 no.8
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• pp.886-891
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• 2008

Mixed connective tissue disease (MCTD) is characterized by diverse symptoms including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, and dermatomyositis, associated with high titers of antibodies to extractable nuclear antigen (ENA), especially anti-ribonucleoprotein (anti-RNP) antibody. Since the first report of 25 cases with MCTD in adults, there have been only a few cases of MCTD reported in children. Here, we report a rare childhood case of MCTD in a 7-year-old girl presenting initially with Raynaud's phenomenon, swollen hands, and ulceration of the right index finger tip followed by alopecia and arthritis during follow-up.

• Korean Journal of Adult Nursing
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• v.12 no.4
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• pp.560-572
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• 2000

This study was designed to offer descriptive data for nursing intervention for relief of fatigue and pain, and to distinguish by the characteristic difference and the symptoms such as fatigue and pain on Ankylosing Spondylitis (AS), Fibromyalgia(FM), and Systemic Lupus Erythematosus(SLE) patients. The sample consisted of 92 patients(AS 29; FM 30; SLE 33) who visited H-University Rheumatism Hospital in Seoul. The data were collected by a structured questionnaire from May 1, 1999 to April 30, 2000. The results were as follows: Patients of 95% experienced fatigue in the last week and a fatigue score of three disease groups were above average. The fatigue score of FM patients was highest in the other disease, but which was not a statistically significant difference(F=1.417, p=.248). The mean score of AS and FM patients in pain was higher than the SLE patients, and there was the statistical significance among the three groups on pain (F=8.239, p=.001). There wasn't a statistical difference among three groups on coping wtih pain(F=1.451, p=.240). There wasn't any correlation between fatigue and pain in each disease (AS: r=.008, p=.966; FM: r=.328, p=.077; SLE: r=.237,p=.185). Therefore, morning stiffness and pain management during sleeping is needed through good body alignment in the AS patients. Adequate rest for fatigue and multiple coping strategies for pain maybe basic nursing intervention in FM and SLE. According to their fatigue rhythm, a regular exercise program is needed for rheumatic disease because they complained of fatigue above average and their fatigue was repeated better and worse only during the one week.