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目的:比较微小病变样Ig A肾病(MCD-Ig AN)与微小病变肾病(MCD)患者的临床病理特点、治疗反应及预后。方法:回顾性分析Ig AN随访登记数据库中80例随访时间≥3年的MCD-Ig AN患者和77例随访时间≥3年的MCD患者的资料。结果:(1)两组患者临床表现相似,以青年男性多见,多数患者以肾病综合征为主要表现,部分患者伴不同程度的镜下血尿,但与MCD患者相比,MCD-Ig AN患者肾活检时基线血清白蛋白、估算的肾小球滤过率(e GFR)更低(P均<0.01),而尿N-乙酰-β-D氨基葡萄糖苷酶、尿视黄醇结合蛋白水平更高(P均<0.01)。(2)肾脏病理:MCD-Ig AN与MCD患者相比,肾小管间质急性和慢性病变均较重(P<0.05)。(3)激素疗效:80例MCD-Ig AN与77例MCD患者激素治疗的疗效比较,8周完全缓解、部分缓解及未缓解均无显著差异(88.8%vs 90.9%,10.0%vs 5.2%,1.3%vs 3.9%,P均>0.05)。达到缓解的中位时间分别是4周(1~24周)和4周(1~28周)。两组患者激素疗效无显著差异。(4)复发和预后:至3年随访期末,MCD-Ig AN患者复发率显著低于MCD患者(45.0%vs 63.6%,P<0.05)。无一例患者进入ESRD,仅2例MCD-Ig AN患者e GFR较基线下降>50%。结论:MCD-Ig AN与MCD患者在临床表现、病理特点、激素治疗的疗效及预后上均无明显差异。值得注意的是,MCD-Ig AN患者伴较显著的肾小管间质病变和损伤。
Objective: To compare the clinicopathological characteristics, treatment response and prognosis of patients with minimal change-like IgA nephropathy (MCD-Ig AN) and minimal change nephropathy (MCD). Methods: Eighty patients with MCD-Ig AN who were followed up for 3 years or more and 77 patients with MCD who were followed up for 3 years were retrospectively analyzed. Results: (1) The clinical manifestations of the two groups were similar and were more common in young males. Most patients had nephrotic syndrome as the main manifestation. Some patients had microscopic hematuria with different degrees. However, MCD-Ig AN patients Baseline serum albumin at renal biopsy showed lower estimated glomerular filtration rate (eGFR) (P <0.01), whereas urinary N-acetyl-β-D-glucosaminidase and urinal retinol binding protein Higher (P <0.01). (2) Renal pathology: Both acute and chronic tubulointerstitial lesions were more severe in MCD-Ig AN and MCD patients (P <0.05). (3) The effect of hormones: The effect of hormone therapy in 80 patients with MCD-Ig AN and 77 patients with MCD was completely relieved in 8 weeks, with no significant difference between partial response and non-response (88.8% vs 90.9%, 10.0% vs 5.2% 1.3% vs 3.9%, P> 0.05). The median time to remission was 4 weeks (1 to 24 weeks) and 4 weeks (1 to 28 weeks), respectively. There was no significant difference in hormonal efficacy between the two groups. (4) Relapse and prognosis: By the end of the 3-year follow-up period, the relapse rate of MCD-Ig AN patients was significantly lower than that of MCD patients (45.0% vs 63.6%, P <0.05). None of the patients entered ESRD, and only 2 patients with MCD-Ig AN had eGFR> 50% lower than baseline. Conclusion: There is no significant difference in clinical manifestations, pathological features, efficacy and prognosis of hormone therapy between MCD-Ig AN and MCD patients. It is noteworthy that MCD-Ig AN patients with more significant tubulointerstitial lesions and injuries.