• 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.

• Tuberculosis and Respiratory Diseases
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• v.62 no.1
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• pp.67-70
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• 2007

A thymic carcinoma is a rare malignant neoplasm of the thymus epithelium, which can be distinguished from a benign or invasive thymoma. Contrary to a thymoma, the association of a thymic carcinoma and autoimmune disease is rare, with only a few cases having been reported. Herein, a case of thymic carcinoma diagnosed concurrently with systemic lupus erythematosus (SLE) is reported. A 49 year-old man presented at our clinic with myalgia. He was diagnosed with SLE, based on an oral ulcer, lymphopenia, and positive ANA and anti-Sm antibodies. Incidentally, a routine chest X-ray showed a large mediastinal mass. Pathological examination of the mediastinal mass revealed an undifferentiated thymic carcinoma, of WHO classification type C. Further work-up for staging showed multiple bone and lung metastases. With a palliative aim, he received systemic chemotherapy, but refused further chemotherapy after the $2^{nd}$ course. Currently, the patient has not been followed up since the chemotherapy.

• Korean Journal of Head & Neck Oncology
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• v.38 no.2
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• pp.23-27
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• 2022

Systemic lupus erythematosus(SLE) is a multisystemic disorder of autoimmune etiology. SLE can occur commonly in young women, and the early symptoms include fever, myalgia, arthralgia, weight loss, lymphadenopathy and these nonspecific symptoms develop into skin rash, splenomegaly, serositis and encephalopathy. Diagnosis of SLE requires clinical and serologic criteria, and treatment choices are hydroxyquinolone and NSAIDs for mild disease, corticosteroids and immunosuppressant for severe disease. In lupus patient, the prevalence of lymphadenopathy is 12~59%. Although lymphadenopathy is common finding in SLE, it is hard to distinguish in early phase of SLE. A 38-year-old woman visited our hospital for cervical lymphadenopathy with polyarthritis and malaise. Multiple cervical lymph nodes enlargement was found on Neck CT, and serologic laboratory test including ANA, antiphospholipid antibody, and anti-dsDNA was positive. For excluding lymphoma, PET-CT and excisional biopsy were performed. The patient finally diagnosed with SLE, and got regular follow-up without complication.

• Journal of Yeungnam Medical Science
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• v.15 no.2
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• pp.371-380
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• 1998

Systemic lupus erythematosus(SLE) is an autoimmune disease which may affect many different organs and disclose various clinical manifestations. Recently central nervous system(CNS) involvement has been recognized as an increasingly significant contributor to morbidity and mortality of SLE. The clinical manifestations of CNS-lupus are highly variable and range from mild cognitive dysfunction, movement disorder, headache, psychosis to life-threatening stroke and coma. Among the neuropsychiatric disorders encountered in patients with SLE, cerebrovascular disease has been a relatively rare complication. The diagnosis and management of CNS-lupus is difficult because of the lack of useful diagnostic methods. If cerebrovascular involvement is suspected, aggressive treatment such as high dose steroid, immunosuppressive therapy, plasma exchange may be required to reduce high mortality rate. We experienced 2 cases cerebrovascular dis eases occurring in SLE patients which presented with various neuropsychiatric manifestations. They were diagnosed as CNS-lupus by neuropsychiatric symptoms, brain MRI, and BEG, and showed good response to high dose steroid pulse therapy.

• 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.

• The Journal of Internal Korean Medicine
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• v.25 no.3
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• pp.573-581
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• 2004

Evans syndrome, as originally described, refers to autoimmune hemolytic anemia accompanied by thrombocytopenia. The autoantibodies in Evans syndrome are directed specifically against red cells, platelets, or neutrophils and are not crossreacting. The incidence of autoimmune hemolytic anemia is estimated to be approximately 10 cases per million people. Many patients have associated disorders, such as lupus erythematosus and other autoimmune disease, chronic lymphadenopathy, or hypogammaglobulinemia. In Oriental Medicine, the approach to Evans syndrome is made in view of deficiency of blood. The ailment was treated through methods of Oriental Medicine. Acupucture and herbal medicine were administered to patients diagnosed with Evans Syndrome by ecchymosis at the lower limb after continued gingival bleeding. As a result significant improvement in RBC, Hb, Hct, and PLT were observed and complaints abated.

• Clinical and Experimental Pediatrics
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• v.50 no.12
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• pp.1180-1187
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• 2007

Systemic lupus erythematosus (SLE) is an episodic, multi-system, autoimmune disease characterized by widespread inflammation of blood vessels and connective tissues and by the presence of antinuclear antibodies (ANAs), especially antibodies to native (double-stranded) DNA (dsDNA). Its clinical manifestations are extremely variable, and its natural history is unpredictable. Untreated, SLE is often progressive and has a significant fatality rate. The most widely used criteria for the classification of SLE are those of the American College of Rheumatology (ACR), which were revised in 1982 and modified in 1997. The presence of four criteria have been diagnosed as a SLE. Rashes are common at onset and during active disease. The oral mucosa is the site of ulceration with SLE. Arthralgia and arthritis affect most children and these symptoms are short in duration and can be migratory. Lupus nephritis may be more frequent and of greater severity in children than in adults. The initial manifestation of nephritis is microscopic hematuria, followed by proteinuria. The most common neuropsychiatric symptoms are depression, psychosis(hallucination and paranoia) and headache. CNS disease is a major cause of morbidity and mortality. Pericarditis is the most common cardiac manifestation. Libman-Sacks endocarditis is less common in children. The most frequently described pleuropulmonary manifestations are pleural effusions, pleuritis, pneunonitis and pulmonary hemorrhage. During the active phase ESR, CRP, gamma globulin, ferritin and anti-dsDNA are elevated. Antibodies to dsDNA occur in children with active nephritis. Antibodies to the extractable nuclear antigens (Sm, Ro/SS-A, La/SS-B) are strongly associated with SLE. Specific treatment should be individualized and based on the severity of the disease. Sepsis has replaced renal failure as the most common cause of death.

• IMMUNE NETWORK
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• v.19 no.1
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• pp.7.1-7.11
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• 2019

Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disorder that affects mainly salivary and lacrimal glands, but its cause remains largely unknown. Clinical data indicating that SS occurs in a substantial proportion of patients with lupus points to common pathogenic mechanisms underlying the two diseases. To address this idea, we asked whether SS develops in the lupus-prone mouse strain sanroque (SAN). Owing to hyper-activation of follicular helper T (Tfh) cells, female SAN mice developed lupus-like symptoms at approximately 20 wk of age but there were no signs of SS at that time. However, symptoms typical of SS were evident at approximately 40 wk of age, as judged by reduced saliva flow rate, sialadenitis, and IgG deposits in the salivary glands. Increases in serum titers of SS-related autoantibodies and numbers of autoantibody-secreting cells in cervical lymph nodes (LNs) preceded the pathologic manifestations of SS and were accompanied by expansion of Tfh cells and their downstream effector cells. Thus, our results suggest that chronic dysregulation of Tfh cells in salivary gland-draining LNs is sufficient to drive the development of SS in lupus-prone mice.

• Clinical and Experimental Pediatrics
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• v.46 no.10
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• pp.1029-1031
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• 2003

Hemophagocytic lymphohistiocytosis is a reactive disorder characterized by a generalized non-malignant histiocytic proliferation with prominent hemophagocytosis by stimulated histiocytes in the bone marrow and reticuloendothelial systems resulting in pancytopenia and liver dysfunction. Several diseases including infection, malignancy and autoimmune disease are known to be causative disorders. This case demonstrated histiocytic hemophagocytosis in the bone marrow, resulting in pancytopenia during treatment of systemic lupus erythematosus and did not show any underlying disease.

• Genomics & Informatics
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• v.14 no.3
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• pp.85-89
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• 2016

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organ systems. Although the etiology of SLE remains unclear, it is widely accepted that genetic factors could be involved in its pathogenesis. A number of genome-wide association studies (GWASs) have identified novel single-nucleotide polymorphisms (SNPs) associated with the risk of SLE in diverse populations. However, not all the SNP candidates identified from non-Asian populations have been validated in Koreans. In this study, we aimed to replicate the SNPs that were recently discovered in the GWAS; these SNPs have not been validated in Koreans or have only been replicated in Koreans with an insufficient sample size to conclude any association. For this, we selected five SNPs (rs1801274 in FCGR2A and rs2286672 in PLD2, rs887369 in CXorf21, rs9782955 in LYST, and rs3794060 in NADSYN1). Through the replication study with 656 cases and 622 controls, rs1801274 in FCGR2A was found to be significantly associated with SLE in Koreans (odds ratio, 1.26, 95% confidence interval, 1.06 to 1.50; p = 0.01 in allelic model). This association was also significant in two other models (dominant and recessive). The other four SNPs did not show a significant association. Our data support that FCGR polymorphisms play important roles in the susceptibility to SLE in diverse populations, including Koreans.