• Clinical and Experimental Pediatrics
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• 제63권8호
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• pp.291-300
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• 2020

Advances in autoimmune encephalitis studies in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to the disorder. The disorder or syndrome has been linked to a wide variety of pathologic processes associated with the neuron-specific autoantibodies targeting intracellular and plasma membrane antigens. However, current criteria for autoimmune encephalitis are quite dependent on antibody testing and responses to immunotherapy, which might delay the diagnosis. This form of encephalitis can involve the multifaceted presentation of seizures and unexpected behavioral changes. The spectrum of neuropsychiatric symptoms in children is less definitive than that in adults, and the incorporation of clinical, immunological, electrophysiological, and neuroradiological results is critical to the diagnostic approach. In this review, we document the clinical and immunologic characteristics of autoimmune encephalitis known to date, with the goal of helping clinicians in differential diagnosis and to provide prompt and effective treatment.

• 대한족부족관절학회지
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• 제23권3호
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• pp.131-134
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• 2019

Stiff-person syndrome is a rare disorder, and the natural history of the syndrome has not been completely ascertained. The symptoms range from mild to severe and can progress over time: the final result can be significant disability. However, this syndrome is often misdiagnosed due to a lack of understanding of the clinical manifestations. We report the case of a patient who presented with slowly progressing gait disturbance and lower extremity pain and was later diagnosed as suffering from stiff-person syndrome. The patient experienced symptomatic improvement after the administration of benzodiazepines. No recurrence of symptoms has been reported. If the characteristic clinical features and electromyography findings of the syndrome are accurately interpreted, diagnosis of patients with abnormal muscle tension can be effectively done.

• 대한세포병리학회지
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• 제13권1호
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• pp.33-37
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• 2002

We report two different types of thyroid lymphoma associated with Hashimoto's thyroiditis. Both showed autoantibodies and were compatible with Hashimoto's thyroiditis according to their clinical backgrounds. A 76-year-old female noted a painless, rapidly growing mass in her neck which was diagnosed as diffuse non-Hodgkin's lymphoma, large cell type, after the fine needle aspiration cytology of the thyroid. She underwent chemo-radiotherapy and is free of the disease 10 months after diagnosis. The other patient, a 73-year-old female with a diffuse golfer, was diagnosed on fine needle aspiration cytology as having Hashimoto's thyroiditis. Three years later she developed a hard nodular growth in the both lobes of the thyroid. This was subjected to fine needle aspiration cytology and needle biopsy and was diagnosed as a MALT lymphoma. She refused any treatment and died 12 months after the diagnosis.

• Journal of Oral Medicine and Pain
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• 제48권3호
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• pp.118-122
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• 2023

In this study, we investigate the emergence of bullous pemphigoid (BP) after the administration of the Severe Acute Respiratory Syndrome-Coronavirus Disease 2019 (SARSCOVID-19) vaccine. The study presents two cases of women, aged 47 and 53, diagnosed with BP following SARS-COVID-19 vaccination. BP is a common autoimmune blistering disorder prevalent among older populations, with an incidence rate ranging from 2 to 40 cases per million individuals. This condition arises when autoantibodies target adhesive proteins in the skin, resulting in blister formation and mucosal erosion. Drug-induced bullous pemphigoid (DIBP) shares similarities with the classic form of BP but may be influenced by medication usage. Notably, DIBP exhibits distinct characteristics, such as affecting a younger demographic and involving mucosal regions more prominently than classic BP. The growing incidence of BP is linked to factors such as an aging population and the rise of drug-induced cases. This case report provides valuable insights into comprehending DIBP, elucidating post-vaccination discomforts, particularly those related to oral lesions and the exacerbation of existing lesions. By elucidating these aspects, we aim to advance the understanding of DIBP within the medical community.

• 대한영상의학회지
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• 제81권2호
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• pp.453-458
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• 2020

하시모토 뇌병증은 다른 대뇌 질환의 증거가 없이 항갑상선 항체의 높은 혈청 농도를 특징으로 하는 드문 자가 면역 질환이다. 하시모토 뇌병증의 자기공명영상 소견은 비특이적이고, 현재까지 미만성 또는 국소 백질 변화가 보고 된 바 있다. 저자는 증가된 항갑상선 항체와 동반하여 수막뇌염과 유사한 영상 소견을 보인 79세 여성의 하시모토 뇌병증을 경험하여 이에 대해 보고하고자 한다. 환자의 일련의 자기공명영상에서 치료 전 관찰되던 여러 T2-고강도 병변과 미만성의 뇌수막 조영증강은 스테로이드 요법 후 소실되었다.

• IMMUNE NETWORK
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• 제22권4호
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• pp.32.1-32.20
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• 2022

Sjögren syndrome (SS) is a chronic autoimmune disorder that primarily targets the salivary and lacrimal glands. The pathology of these exocrine glands is characterized by periductal focal lymphocytic infiltrates, and both T cell-mediated tissue injury and autoantibodies that interfere with the secretion process underlie glandular hypofunction. In addition to these adaptive mechanisms, multiple innate immune pathways are dysregulated, particularly in the salivary gland epithelium. Our understanding of the pathogenetic mechanisms of SS has substantially improved during the past decade. In contrast to viral infection, bacterial infection has never been considered in the pathogenesis of SS. In this review, oral dysbiosis associated with SS and evidence for bacterial infection of the salivary glands in SS were reviewed. In addition, the potential contributions of bacterial infection to innate activation of ductal epithelial cells, plasmacytoid dendritic cells, and B cells and to the breach of tolerance via bystander activation of autoreactive T cells and molecular mimicry were discussed. The added roles of bacteria may extend our understanding of the pathogenetic mechanisms and therapeutic approaches for this autoimmune exocrinopathy.

• Korean Journal of Radiology
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• 제25권9호
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• pp.807-823
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• 2024

Autoimmune encephalitis (AE) is a category of immune-mediated disorders of the central nervous system (CNS) affecting children and adults. It is characterized by the subacute onset of altered mentation, neurocognitive issues, refractory seizures/drug-resistant epilepsy, movement disorders, and/or autonomic dysfunction. AE is mediated by autoantibodies targeting specific surface components or intracytoplasmic antigens in the CNS, leading to functional or structural alterations. Multiple triggers that induce autoimmunity have been described, which are mainly parainfectious and paraneoplastic. The imaging features of AE often overlap with each other and with other common causes of encephalitis/encephalopathy (infections and toxic-metabolic etiologies). Limbic encephalitis is the most common imaging finding shared by most of these entities. Cortical, basal ganglia, diencephalon, and brainstem involvement may also be present. Cerebellar involvement is rare and is often a part of paraneoplastic degeneration. Owing to an improved understanding of AE, their incidence and detection have increased. Hence, in an appropriate setting, a high degree of suspicion is crucial when reporting clinical MRIs to ensure prompt treatment and better patient outcomes. In this review, we discuss the pathophysiology of AE and common etiologies encountered in clinical practice.

• IMMUNE NETWORK
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• 제14권1호
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• pp.38-44
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• 2014

K/BxN serum can transfer arthritis to normal mice owing to the abundant autoantibodies it contains, which trigger innate inflammatory cascades in joints. Little is known about whether gut-residing microbes affect host susceptibility to autoantibody-mediated arthritis. To address this, we fed C57BL/6 mice with water containing a mixture of antibiotics (ampicillin, vancomycin, neomycin, and metronidazol) for 2 weeks and then injected them with K/BxN serum. Antibiotic treatment significantly reduced the amount of bacterial genomic DNA isolated from fecal samples, in particular a gene encoding 16S ribosomal RNA derived from segmented filamentous bacteria. Arthritic signs, as indicated by the arthritic index and ankle thickness, were significantly attenuated in antibiotic-treated mice compared with untreated controls. Peyer's patches and mesenteric lymph nodes from antibiotic-treated mice contained fewer IL-17-expressing cells than those from untreated mice. Antibiotic treatment reduced serum C3 deposition in vitro via the alternative complement pathway. IL-$17^{-/-}$ congenic C57BL/6 mice were less susceptible to K/BxN serum-transferred arthritis than their wild-type littermates, but were still responsive to treatment with antibiotics. These results suggest that gut-residing microbes, including segmented filamentous bacteria, induce IL-17 production in GALT and complement activation via the alternative complement pathway, which cause the host to be more susceptible to autoantibody-mediated arthritis.

• Tuberculosis and Respiratory Diseases
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• 제44권4호
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• pp.922-929
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• 1997

저자들은 호흡곤란으로 내원한 환자에서, 외이, 코, 기관과 기관지를 침범한 재발성 다발성 연골염 1예를 경험하였기에 보고하는 바이다.

• Tuberculosis and Respiratory Diseases
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• 제67권6호
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• pp.569-573
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• 2009

Idiopathic pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by surfactant component accumulation in the alveolar space. Idiopathic PAP has recently been recognized as a autoimmune disease of impaired alveolar macrophage function caused by autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF). While whole lung lavage has been the standard treatment, not every patient shows a complete response. Subcutaneous injection or inhalation of GM-CSF is another promising treatment option for PAP. A 45-year-old patient visited our hospital for dyspnea, he was diagnosed as PAP and underwent whole lung lavage. Eighteen months later, the patient had not achieved complete remission in despite of initial response. After then he was administered with GM-CSF (5 ${mu}g/kg/day$, subcutaneous injection) for fivetimes a week during 2 months. Nine months later, the abnormal shadows in high-resolution computed tomography (HRCT) decreased and the patient fully recovered in forced vital capacity. After 60 months, the HRCT scan showed complete remission of PAP.