微小病变样IgA肾病临床特点分析  

Characteristics of Ig A nephropathy with minimal change lesions

作　　者：李小伟[1,2] 乐伟波[2] 梁少珊[2] 成水芹[2] 曾彩虹[2] 王金泉[2] 刘志红[1,2] 

机构地区：[1]东南大学临床医学院,南京210009 [2]南京军区南京总医院囝家肾脏疾病临床医学研究中心全军肾脏病研究所,南京210016

出　　处：《中国实用内科杂志》2015年第9期767-771,共5页Chinese Journal of Practical Internal Medicine

基　　金：国家重点基础研究发展计划(2012CB517606);国家自然科学基金重大国际合作基金(81020108016)

摘　　要：目的分析微小病变样Ig A肾病(MCD-IgAN)患者临床病理特点、治疗反应及长期预后。方法回顾性分析1989年1月至2013年7月南京军区南京总医院Ig A肾病随访登记数据库中经肾活检确诊的247例MCD-IgAN患者,并与该数据库中的1155例Non-MCD-IgAN患者进行比较。结果 (1)MCD-IgAN患者306例,占同期Ig A肾病患者的2.57%(306/11885)。(2)对资料完整的247例MCD-IgAN患者进行分析,其中男167例(67.6%),女80例(32.4%);平均年龄(27.05±11.03)岁。81.4%患者表现为肾病综合征,18.7%患者伴镜下血尿,19.0%患者出现高血压,出现低钠血症、血栓、急性肾损伤者分别为27.5%、2.4%、8.9%。(3)肾脏病理:34例(13.7%)患者出现中、重度的肾小管间质急性病变,11例(4.5%)患者出现轻度的肾小管间质慢性病变。(4)治疗反应:激素治疗8周完全缓解、部分缓解和无效的患者分别是216例(87.4%)、21例(8.5%)和10例(4.0%)。(5)预后:MCD-IgAN患者中没有进入ESRD者仅4例(1.6%)进入肾脏终点事件。与Non-MCD-IgAN患者108例(9.4%)进入ESRD,163例(14.1%)进入肾脏终点事件相比,MCD-IgAN患者肾脏预后好(P<0.05)。结论 MCD-IgAN患者与Non-MCD-IgAN患者相比,对激素治疗反应好,总体预后好。临床上需重视这组特殊类型IgAN的诊断和治疗。Objective： To analyze the clinic-pathological characteristics, treatment response and long-term prognosis of IgA nephropathy with minimal change disease （MCD-IgAN）. Methods Patients with biopsy-proven MCD-IgAN from the IgA nephropathy Registry of Jinling Hospital were systematically reviewed and compared with another 1155 patients with Non-MCD- IgAN. Results A total of 306 patients were diagnosed with MCD-IgAN, which accounted for 2.57% of IgA nephropathy during the same period. Totally 247 cases wRh complete data were enrolled in this study. Male predominance （67.6%） was observed in patients with MCD-IgAN, with an average age of （27.05 ± 11.03） years at renal biopsy. Initial clinical features included： nephrotic syndrome （81.4%）, microhematuria （18.7%）, hypertension （19.0%）, hyponatremia （27.5%）, thrombus （2.4%） and acute kidney injury （8.9%）. Pathological data revealed that 34 cases （13.7%） had moderate to severe acute tubulointerstitial lesions and 11 cases （4.5%） had mild chronic tubulointerstitial lesions. After 8 weeks of corticosteroid therapy; a complete remission was attained in 216 patients （87.4%）, a partial remission in 21 patients （8.5%）, and no remission in 10 patients （4.0%）. In MCD-IgAN group, no patients entered end stage renal disease （ESRD） and only 4 patients （1.6%） had greater than 50% reduction of epidermal growth factor receptor （eGFR）. In Non- MCD-IgAN group, 108 patients （9.4%） entered ESRD and 163 patients （14.1%） had greater than 50% reduction ofeGFR. Patients with MCD-IgAN had significantly better renal outcome than those with Non-MCD-IgAN （P 〈0.05）. Conclusion Compared with Non-MCD-IgAN patients, those with MCD-IgAN have better response to corticosteroid therapy and better renal outcome. A greater awareness of this rare entity ofIgA nephropathy needs to be created to facilitate proper diagnosis and treatment.

关 键 词：IGA肾病 肾小球微小病变 肾病综合征 

分 类 号：R692[医药卫生—泌尿科学]