| Update on the pathophysiology of systemic lupus erythematosus |
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Eun Ha Kang |
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가톨릭대학교 의과대학 1내과학교실, 2진단방사선과교실, 3병원병리과교실 |
| 전신 홍반 루푸스의 병태 생리의 최신지견 |
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강은하 |
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Departments of 1Internal Medicine, 2Radiology and 3Hospital Pathology, The Catholic University of Korea College of Medicine, Seoul, Korea |
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| Abstract |
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Systemic lupus erythematosus (SLE) is a systemic inflammatory disease in which all the key components of the immune system are involved to break immune tolerance and to cause tissue inflammation. Breach of immune tolerance as evidenced by the appearance of autoantibodies is observed long before disease onset. Candidate gene analyses or genome-wide scans suggest that multiple genetic loci affecting both innate and adaptive immunity are involved in determining disease susceptibility. These genetic evidences have been further supported by abnormalities found in the cells and molecules influenced by these genes. Autoantigen accumulation by ineffective clearance of apoptotic cells due to impaired innate immune system provides persistent antigenic stimuli to expand and differentiate autoreactive T cells. Certain HLA genes further promote autoreactive T cell expansion by affecting either T cell receptor-peptide-MHC interactions or T cell repertoire selection. Defects in T cell signaling render them hyper-responsive to antigen stimuli and resistant to activation-induced cell death. B cell signaling defects help autoreactive B cells escape into periphery, lower activation threshold, and prolong survival. Pathogenic autoantibodies are produced as a result of accumulation of these abnormalities, followed by immune complex formation, complement fixation, and finally tissue damage. This review will discuss in detail the series of events involved in the break-down of immune tolerance in SLE. (Korean J Med 78:403-408, 2010) |
| Key Words:
Systemic lupus erythematosus; Pathophysiology; Autoimmunity |
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