遗传病的疾病。

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铃木黑川纪章铃木M, N

遗传病的疾病。

地中海Exp。2004年9月,4 (1):10 - 20。

PubMed ID

15598081 (在PubMed

]

文摘

遗传病疾病(BD)是一种系统性疾病的复发性急性炎症,表现为口腔溃疡的主要症状,葡萄膜炎,皮肤损伤和生殖器溃疡。参与肠道、船只和中枢神经系统(CNS)有时会导致预后不良。BD患者已知分布沿着古老的丝绸之路。发病率相对更高的从东亚到地中海地区约1 - 10的病人10000人,而只有1 - 2患者有1000000人在英国和北美。虽然疾病的病因仍未知,HLA-B51患病率高,增加表达热休克蛋白60和Th1主导免疫反应的患者被认为是重要的发病机制。非中性粒细胞激活和感染链球菌sanguis和单纯疱疹病毒也会相关联。因为BD缺乏任何特殊的症状和实验室研究结果,提出的诊断主要依赖于标准的国际研究小组1990年遗传病疾病。在日本,诊断也在1987年根据日本标准修订。最近,日本的遗传病病研究委员会再次修订了2003年日本标准,以避免过度诊断。介绍了日本新标准审查。 Differential diagnosis excluding Sweet's disease, pemphigus, erythema nodosum and Crohn's disease is important, and positive laboratory data for pathergy test, prick test for dead Streptococci and HLA-B51 are emphasized to make appropriate diagnosis in these criteria. Pathological findings of the disease-affected site such as erythematous nodosum is also stressed. Treatment for the disease has been chosen according to the clinical symptoms. Non-steroidal anti-inflammatory drugs, immunosuppressants, corticosteroids and colchicine are basically introduced. Recently, effects of interferon-alpha/beta, anti-tumor necrosis factor antibody and thalidomide are encouraging, specifically in treatment for the cases with poor prognosis including eye, intestine, vessel and CNS involvement. Low dose weekly administration of methotraxate looks effective for the cases with CNS involvement. Further studies for elucidation of the etiology, improvement of the diagnostic criteria and development of new therapy are needed to conquer the disease.

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