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Secondary systemic lupus erythematosus: an analysis of 4 cases of uncontrolled hereditary angioedema.

机译:继发性系统性红斑狼疮:4例不受控制的遗传性血管性水肿的分析。

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摘要

The association of systemic lupus erythematosus and hereditary angioedema (HAE) has formed the basis of numerous case reports and is hypothesised to result from consumption of complement C4 with consequent impaired clearance of apoptotic cells. We describe the development of frank lupus or lupus-like syndrome in four HAE patients with uncontrolled angioedema and low levels of serum C4. Measures that limit hypocomplementaemia in HAE may reduce the incidence of secondary SLE.
机译:系统性红斑狼疮与遗传性血管性水肿(HAE)的关联已形成众多病例报告的基础,并被认为是由于食用补体C4导致凋亡细胞清除受损而导致的。我们描述了四名患有不受控制的血管性水肿和血清C4水平低的HAE患者的坦率性狼疮或类狼疮综合征的发展。限制HAE中低补体血症的措施可降低继发性SLE的发生率。

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