전신성 홍반성 루푸스 = Systemic Lupus Erythematosus

다국어 초록 (Multilingual Abstract)

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the

production of autoantibodies to components of the cell nucleus with diverse clinical

manifestations, predominantly in women during reproductive years. SLE is caused by interaction

between susceptibility genes and environmental factors, which result in abnormal immune

response. SLE is caused by failure in regulating the production of pathogenic autoantibodies and

the formation of immune complex. Abnormalities in the immune response regulation display a

decreased ability to clear immune complexes and apoptotic cells. Genetic predisposition to SLE

involves multiple genes. Genetic variants predisposing to SLE may influence clearance of

immune complexes or apoptotic bodies, activation of B cells or T cells, and inflammation related

to dendritic cell activation. Environmental factors that predispose to or activate SLE include

ultraviolet B light, infection with Epstein-Barr virus, female gender, and exposure to estrogencontaining

medications. The diagnosis of SLE is based on characteristic clinical features and

autoantibodies. The diagnostic criteria of the American College of Rheumatology (ACR) reflect

the major clinical features of the disease (mucocutaneous, articular, serosal, renal, neurologic)

and incorporate the associated laboratory findings (hematologic and immunologic). Four or more

criteria are required for diagnosis. There is no effective cure for SLE, and complete sustained

remissions are rare. The treatment should be tailored based on the clinical manifestations in an

individual patient. Conservative therapies for management of non-life-threatening manifestations

of SLE comprise NSAIDs, corticosteroids and antimalarials. Treatment of severe organ damage

requires immunosuppressive agents. Targeted biologic therapies are under development and

appear to be promising.

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