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嗜酸性肉芽肿性多血管炎并发肾脏受累的临床特征

张上珠 杨宏仙 费允云 张文 赵岩 张奉春 曾小峰

中华临床免疫和变态反应杂志2017,Vol.11Issue(3):247-252,6.
中华临床免疫和变态反应杂志2017,Vol.11Issue(3):247-252,6.DOI:10.3969∕j.issn.1673-8705.2017.03.009

嗜酸性肉芽肿性多血管炎并发肾脏受累的临床特征

Clinical Features of Eosinophilic granulomatous with polyangiitis patientsc ombined with renal involvement

张上珠 1杨宏仙 2费允云 1张文 1赵岩 1张奉春 1曾小峰1

作者信息

  • 1. 中国医学科学院北京协和医学院北京协和医院风湿免疫科风湿免疫病学教育部重点实验室,北京100730
  • 2. 首都儿科研究所附属儿童医院肾内科
  • 折叠

摘要

Abstract

Objective To investigate the clinical characteristics and laboratory findings of eosino-philic granulomatous with polyangiitis ( EGPA) patients with renal involvement. Methods This study retrospectively collected cases of EGPA inpatients in Peking Union Medical College Hospital during 2000 to 2017. Descriptive analysis and literature review were performed. Results Among the 73 pa-tients, 17 patients ( 23. 3%) experienced varying degrees of renal involvement. Two patients presented with renal involvement as the initial symptom. Among the 17 patients, 12 were men. The average onset age was (43. 5±14. 1) years old. The duration of the disease was (51. 5±92. 9) months. Fifteen cases presented with nasal involvement, 13 cases with asthma, 13 cases with peripheral nerve involvement,12 cases with pulmonary lesion, 7 cases with skin lesion, 4 cases with cardiac involvement, 3 cases with gastrointestinal involvement and 2 with central nerve system involvement. Fourteen patients had positive serum ANCA, which was significantly more frequent than that in patients without renal involve-ment (82. 4% vs. 20. 8%, P<0. 001). Kidney involvements:Fifteen patients presented with hematuri-a, 14 cases with proteinuria, 8 cases with elevated creatinine level and 3 cases with oliguria or anuria. Two patients underwent renal biopsy, and the pathology finding was focal segmental glomerular necrosis with crescent formation. Fifteen patients were treated with high-dose glucocorticoid and cyclophosphamide with good effects. Conclusions EGPA patients may have renal involvement, usually presents with hematuria and proteinuria, with a small number of progressive glomerulonephritis. EGPA patients with renal involvement are frequently ANCA positive. The treatment with glucocorticoid and cy-clophosphamide is effective.

关键词

嗜酸性肉芽肿/多血管炎/肾脏受累/抗中性粒细胞胞浆抗体

Key words

eosinophilic granulomatous with polyangiitis/renal involvement/antineutrophil cytoplasmic antibody

分类

医药卫生

引用本文复制引用

张上珠,杨宏仙,费允云,张文,赵岩,张奉春,曾小峰..嗜酸性肉芽肿性多血管炎并发肾脏受累的临床特征[J].中华临床免疫和变态反应杂志,2017,11(3):247-252,6.

中华临床免疫和变态反应杂志

OACSTPCD

1673-8705

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