伴有新月体形成的原发性IgA肾病的临床与病理特点及预后分析

目的观察伴有新月体形成的原发性IgA肾病的临床、病理特点，分析其对激素及环磷酰胺治疗的反应。 方法收集包头医学院第一附属医院1997年8月至2015年04月收治的80例经肾活检确诊为原发性IgA肾病并伴新月体形成的患者，并依据新月体累及的肾小球比例进行分组，新月体占受累肾小球比例≥50% (A组) 24例;新月体累及的肾小球比例<50%(B组) 56例。肾小球系膜增生、肾小管间质病变采用R.Katafuchi标准积分量化。对两组的临床及病理特点进行比较。治疗方案：将A、B两组再分为单纯糖皮质激素(激素)治疗组，激素＋环磷酰胺治疗组，分别比较不同治疗方案对各组的疗效。A、B组各有24例患者接受了随访。应用SPSS软件进行统计学分析。 结果①临床方面：32例(40%)患者有镜下血尿＋蛋白尿，76例(95%)患者尿蛋白≥2 g/24 h，32例(40%)患者有肉眼血尿；水肿、高血压、肾功能异常者超过半数。A组尿蛋白量及血清肌酐明显高于B组(t＝1.890，t＝2.570; P<0.05)，血清白蛋白及肾小球滤过率明显低于B组(t＝2.681, t＝3.014；P<0.05)。②病理方面：所有受累肾小球的新月体面积百分比为5.92%～88.9%,其中A组为52.6%～88.9%, B组为5.92%～48.9%;与B组比较A组肾小管间质损害更严重，两组比较差异有统计学(P<0.05)。③治疗情况：A组及B组经激素或激素＋环磷酰胺治疗后，尿蛋白定量均明显减少(P<0.05) ；单纯激素治疗后A组血清肌酐较治疗前有明显下降(t＝3.243，P<0.05)。随访2～4年时，A组8例患者出现血清肌酐升高，达透析指征，1例死亡；B组2例患者出现血清肌酐升高(1例原有轻度升高，1例新出现血清肌酐升高)。 结论IgA肾病患者随着新月体占受累肾小球比例的增加，肾小管间质病理损害及临床表现亦逐渐加重且预后不佳；激素治疗可减少伴有大新月体形成的原发性IgA肾病的蛋白尿并有可能改善其肾功能。

Analysis of clinical and pathological features and prognosis for primary IgA nephropathy with glomerular crescents formation

ObjectiveTo observe the clinical and pathological features of primary IgA nephropathy with glomerular crescents formation, and to analyze its response to treatment with corticosteroids and cyclophosphamide. MethodsFrom August 1997 to April 2015 in the First Hospital Affiliated to Baotou Medical College, a total of 80 patients of primary IgA nephropathy with crescents formation confirmed by renal biopsy were divided into group A (area ratio of crescents to involved glomeruli ≥50%) (n=28) and group B (area ratio of crescents to involved glomeruli <50%) (n=52). Glomerular mesangial proliferation and renal tubular interstitial lesions were quantified with scores by the Katafuchi criteria. And the clinical and pathological features were also compared. Treatment plan: both group A and group B were divided into corticosteroids subgroup, and corticosteroids plus cyclophosphamide subgroup. Follow-up was made in group A and group B with 24 cases each. And statistical analysis was made with the SPSS software. ResultsMicroscopic hematuria and proteinuria occurred in 32 patients (40%); 76 patients (95%) had proteinuria no less than 2 g/24 h; 32 patients (40%) had macrohematuria; edema, hypertension, and abnormal kidney function were found in more than 50% of the patients. In group A, proteinuria was significantly higher than in group B (t=1.890, P<0.05), while serum albumin and glomerular filtration rate were significantly lower than in group B (t=2.681, t=3.014; P<0.05). The area percentage of the crescents over the affected glomeruli was 5.92%-88.9% totally, 52.6%-88.9% in group A, and 5.92%- 48.9% in group B. Compared with group B, group A had more serious renal tubular damage (P<0.05). After treatment with corticosteroids plus cyclophosphamide, quantitative proteinuria was significantly reduced in both group A and group B (χ²=4.010, χ²=4.902, P<0.05). After the corticosteroids therapy, the reduction of serum creatinine was more significant in group A than in group B (χ²=5.882, P<0.05). After followed-up for 2-4 years, 8 cases had elevated serum creatinine that met the dialysis indications, and 1 case died in group A; 2 cases had increased serum creatinine (one case with slight baseline elevation, and the other with new elevation of serum creatinine). ConclusionsIn the patients with IgA nephropathy, with increase of the proportion of crescents area over the affected glomeruli, the renal tubulointerstitial pathological damage and clinical manifestation gradually aggravated with poor prognosis. In the primary IgA nephropathy with big crescents formation, the corticosteroids therapy could decrease proteinuria and might be able to improve the renal function.