Boccon-Gibod L, Just J, Grimfeld A, Tournier G
Arch Anat Cytol Pathol. 1989;37(4):177-80.
A six year old girl developed severe pulmonary hemorrhage following appendectomy. Clinical findings and results of immunfluorescence studies of skin, lung and renal biopsy specimens were consistent with systemic lupus erythematosus (SLE). A sister also had hemoptyses and a lupus syndrome. Both girls, but none of the other siblings or parents, were shown to have a partial C4 deficiency. A review of the literature disclosed 13 pediatric cases of SLE with pulmonary hemorrhages. In none of these cases was congenital C4 deficiency reported (although C4 deficiency is known to induce autoimmune diseases, including lupus). Our two patients are therefore the first reported cases of SLE with pulmonary hemorrhage and a partial congenital C4 deficiency.
一名六岁女孩在阑尾切除术后出现严重肺出血。皮肤、肺和肾活检标本的临床发现及免疫荧光研究结果与系统性红斑狼疮（SLE）相符。其妹妹也有咯血及狼疮综合征。两个女孩均被证实存在部分C4缺乏，而其他兄弟姐妹及父母均无此情况。文献回顾发现13例小儿SLE合并肺出血病例。这些病例中均未报告先天性C4缺乏（尽管已知C4缺乏可诱发自身免疫性疾病，包括狼疮）。因此，我们的两名患者是首例报告的SLE合并肺出血及部分先天性C4缺乏的病例。
