Welch T R, McAdams A J, Beischel L S
Children's Hospital Research Foundation, Cincinnati, Ohio, USA.
Arthritis Rheum. 1995 Sep;38(9):1333-7. doi: 10.1002/art.1780380923.
A 15-year-old girl with complete C4 deficiency and a lupus-like disorder developed evidence of nephritis after 4 years of followup. Renal biopsy demonstrated an immune complex glomerulonephritis, with deposits in the capillary loops, the paramesangium, and the mesangial matrix. Renal function was normal. The patient was treated with monthly infusions of intravenous immunoglobulin for 6 months. The treatment was well tolerated, and resulted in resolution of the rash and hematuria. Followup biopsy showed less proliferation and fewer loop deposits. In light of the serious risk of infections that is associated with complement deficiency, approaches to glomerulonephritis that do not include immunosuppression should be considered.
一名患有完全性C4缺乏症和狼疮样疾病的15岁女孩在随访4年后出现了肾炎迹象。肾活检显示为免疫复合物性肾小球肾炎，在毛细血管袢、系膜旁区和系膜基质中有沉积物。肾功能正常。该患者接受了为期6个月的每月一次静脉注射免疫球蛋白治疗。治疗耐受性良好，皮疹和血尿得到缓解。随访活检显示增殖减少，袢内沉积物减少。鉴于与补体缺乏相关的严重感染风险，应考虑不包括免疫抑制的肾小球肾炎治疗方法。
