Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India.
Division of Allergy and Immunology, Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India.
Immunobiology. 2020 Sep;225(5):152000. doi: 10.1016/j.imbio.2020.152000. Epub 2020 Aug 11.
Atypical hemolytic uremic syndrome (aHUS) is caused mainly by complement dysregulation. Although various defects in the complement system explaining pathophysiology have been described in recent years, the etiology still remains unclear in about thirty percent of cases. In exploring other causes, similar to anti- complement factor H (anti-CFH) antibody associated HUS, we hypothesized that anti-complement factor I (anti-CFI) antibody could play a role in aHUS. Further, we tried to describe the clinical profile and outcome of those with high anti CFI antibody titers. Eleven of thirty five children (31 %) diagnosed with aHUS from July 2017 to December 2018 had high IgG anti-CFI antibody titers. Median age was 10 months (6, 33) with no sex difference. Thirty-six percent (4/11) had nephrotic-range proteinuria. C3 was low in 8 children (72.7 %) with mean C3 (68.1 ± 14.7 mg/dL). Plasmapheresis was done in 2 children who promptly responded, suggesting the possible role of anti-CFI antibody in pathogenesis of aHUS in these patients. Further studies examining role of anti-CFI antibodies in aHUS is warranted with longitudinal and genetic studies.
非典型溶血尿毒症综合征 (aHUS) 主要由补体失调引起。尽管近年来描述了许多补体系统缺陷解释其病理生理学的情况，但仍有约 30%的病例病因不明。在探索其他原因时，类似于抗补体因子 H (anti-CFH) 抗体相关的 HUS，我们假设抗补体因子 I (anti-CFI) 抗体可能在 aHUS 中起作用。此外，我们试图描述具有高抗 CFI 抗体滴度的患者的临床特征和结局。2017 年 7 月至 2018 年 12 月，我们诊断了 35 名儿童中的 11 名（31%）患有 aHUS，他们的 IgG 抗 CFI 抗体滴度较高。中位年龄为 10 个月（6，33），无性别差异。36%（4/11）有肾病范围蛋白尿。8 名儿童（72.7%）的 C3 降低，平均 C3（68.1±14.7mg/dL）。2 名儿童接受了血浆置换治疗，反应迅速，提示抗 CFI 抗体可能在这些患者的 aHUS 发病机制中起作用。需要进一步研究抗 CFI 抗体在 aHUS 中的作用，包括进行纵向和遗传研究。
