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肾脏病与透析肾移植杂志 ›› 2017, Vol. 26 ›› Issue (1): 31-36.DOI: 10.3969/cndt.j.issn.1006-298X.2017.01.006

• 论文 • 上一篇    下一篇

血清抗中性粒细胞胞质抗体阳性狼疮性肾炎患者的临床病理特征及预后

  

  • 出版日期:2017-02-28 发布日期:2017-02-21

Clinicopathological characteristics and outcome of lupus nephritis with antineutrophil cytoplasmic antibody positivity

  • Online:2017-02-28 Published:2017-02-21

摘要:

目的:回顾性分析血清抗中性粒细胞胞质抗体(ANCA)阳性狼疮性肾炎(LN)患者的临床、病理特征及预后。
方法:收集本中心1985年1月至2008年12月间明确诊断为血清ANCA阳性LN患者,回顾性分析其临床、病理特征及预后,对照组选取同期诊断为血清ANCA阴性LN患者。
结果:符合入选标准的49例患者中,38例MPOANCA阳性,7例PR3ANCA阳性,4例MPOANCA和PR3ANCA双阳性。女性40例、男性9例。中位年龄33岁。ANCA阳性LN患者,尿沉渣红细胞计数、血清肌酐、SLEDAI评分均高于对照组(P<005);病理类型以LNⅣ型为主,占6122%;光镜下AI、CI评分均显著高于对照组(P<005)。ANCA阳性LN患者中,20例接受吗替麦考酚酸酯(MMF)诱导治疗,18例接受静脉环磷酰胺(CYC)诱导治疗,其余11例患者接受雷公藤多苷等治疗MMF组的完全缓解率(9000% vs 5556%, P=0027)和肾脏生存率(P=0039)均优于CYC组。ANCA阳性LN患者中位随访8783月,有7例患者(1429%)发生终末期肾病,均为MPOANCA阳性。
结论:血清ANCA阳性LN患者肾脏表现为大量的血尿、肾功能损害明显。这部分患者接受MMF诱导治疗,其完全缓解率和肾脏预后均优于CYC。

关键词: 狼疮性肾炎, 抗中性粒细胞胞质抗体, 临床表现, 病理特征, 治疗预后

Abstract:

Objective:To assess the clinical features,pathological presentation and outcome of lupus nephritis(LN) with antineutrophil cytoplasmic antibody(ANCA) positive.
Methodology:The pathology database at our institution was searched for cases that showed LN with ANCA positivity between January 1985 to December 2008. Their data were analyzed and compared with the control group.
Results:The cohort consisted of 40 female and 9 male with an average age of 33 years at biopsy,including 38 MPOANCA,7 PR3ANCA and 4 with double positive. LN with ANCA positive exhibited higher hematuria,serum creatinine and SLEDAI score. On pathological evaluation,Class IV LN predominance was observed,accounting for 6122%. Light microscopy revealed significantly higher scores of AI and CI. Patients receiving mycophenolate mofetil (MMF) as induction therapy had higher complete remission and better renal outcome than those receiving cyclophosphamide. During followup,endstage renal disease developed in 7 (1429%) patients,all of whom were MPOANCA positive.
Conclusion:LN with ANCA positivity was characterized by massive hematuria,advanced renal insufficiency. Higher complete remission and better prognosis were observed when using MMF than cyclophosphamide as induction therapy.