期刊
LUPUS
卷 19, 期 9, 页码 1096-1106出版社
SAGE PUBLICATIONS LTD
DOI: 10.1177/0961203310373370
关键词
C1q; C1r; C1s; C2; C4; complement assays; complement deficiency; CR1; cutaneous lupus erythematosus; hereditary angioedema; lupus erythematosus; mannose-binding lectin
类别
In this review we address the main cutaneous manifestations and diseases associated with deficiencies in components of the complement system. The first part is devoted to hereditary angioedema, in which acute, sometimes life-threatening recurrent attacks of acute swelling, usually associated with gastrointestinal symptoms, occur. It is related to a structural or functional deficiency of C1 esterase inhibitor. Patients usually have lowered C4 levels, and diagnosis relies on determination of antigenic and/or functional C1 inhibitor level. The second part focuses on lupus erythematosus, as deficiencies in early components of the complement system, such as C1q, C1r, C1s, C2 or C4, are the strongest known disease susceptibility genes for the development of human systemic lupus erythematosus. Severe infections early in life and marked photosensitivity in a patient with lupus erythematosus are clues to an underlying complement deficiency. The genetic background and the clinical associations of the different components of the complement system will be detailed. Lupus (2010) 19, 1096-1106.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据