合并毛细血管襻纤维素样坏死的原发性IgA肾病患者的预后

目的 初步探讨合并毛细血管襻纤维素样坏死的IgA肾病(IgAN)的临床病理特点及纤维素样坏死对IgAN近期预后的影响。方法 回顾分析1997年至2004年10月在北京大学第一医院肾内科经肾活检诊断资料完整的780例原发性IgAN。在46例伴有纤维素样坏死的患者中，35例有完整的随访数据，平均随访时间26个月。随机选取同期的80例无毛细血管襻纤维素样坏死的IgAN患者，平均随访时间39个月，比较两组患者的临床病理及预后情况。终点事件定义为Scr上升50%或Ccr下降33%或ESRD。采用Kaplan-Meier方法进行生存分析及Cox风险比例模型筛选预后危险因素。结果 坏死组肾组织的淋巴单核细胞浸润更为明显(P = 0.004)；肾小球硬化比率较低(P = 0.002)。坏死组中仅有2例以急性肾功能衰竭(ARF)起病，3例达随访终点；80例非坏死组患者中有14例达终点。多因素分析提示，纤维素样坏死不是IgAN预后的独立危险因素，而严重慢性化病变是唯一提示预后不良的危险因素(RR = 23.13， P < 0.01)。结论 合并纤维素样坏死的IgAN在临床病理方面并无显著特点，纤维素样坏死不影响IgAN的短期预后。

Necrotizing lesions in IgA nephropathy is not indicative of unbeneficial effect in prognosis

Objective To study the prognosis of IgA nephropathy(IgAN). Methods Forty-six out of 780 IgAN patients biopsied in a single unit of north China since 1997 showed segmental necrotizing lesions. Thirty-five of these patients were followed up for (26±26) months after biopsy. Their morphological features and natural history were compared with those of control group of 80 patients without segmental necrosis， who had comparable serum creatinine with a follow-up for (39±23) months. Progression was indicated as 50% elevation of serum creatinine or 33% decline of Ccr or development of ESRD. Their clinical outcomes were compared using Kaplan-Meier estimation. Cox regression was performed to identify predictive factors for clinical events. Results Only 2 necrotizing patients showed ARF. No difference was found in the clinical symptoms presented. The necrotizing group showed a more significant accumulation of monocytes and lymphocytes (P = 0.004) with lower percentage of glomerular sclerosis (P = 0.002). Among the groups of follow-up shorter than 24 months， 3 of 22 necrotizing IgAN patients and 1 of 28 non-necrotizing IgAN patients had futher progression (P = 0.08). In other groups， all necrotizing IgAN patients were event-free， 13 of 52 non-necrotizing IgAN patients progressed， in which necrotizing lesion was not an independent risk factor. Multivariate analysis showed that lesions with severe chronic background were the only prognostic factors (RR = 23.13， P < 0.01)， indicating prognosis was associated with the severity of universal lesions， not with necrotizing lesions alone. Conclusions Based on the present data， necrotizing lesion is not an independent risk factor and may not require aggressive therapy. Longer period of follow-up is needed for further confirmation.