Khan Sujoy, Tarzi Michael D, Doré Philip C, Sewell W A C, Longhurst Hilary J
Path Links Immunology, Scunthorpe General Hospital, Scunthorpe, North Lincolnshire, UK.
Clin Immunol. 2007 Apr;123(1):14-7. doi: 10.1016/j.clim.2006.09.015. Epub 2006 Nov 13.
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.
系统性红斑狼疮与遗传性血管性水肿（HAE）的关联构成了众多病例报告的基础，据推测这是由于补体C4的消耗以及随之而来的凋亡细胞清除受损所致。我们描述了4例血管性水肿未得到控制且血清C4水平较低的HAE患者出现明显狼疮或狼疮样综合征的情况。限制HAE患者低补体血症的措施可能会降低继发性系统性红斑狼疮的发病率。
