狼疮性肾炎的病理刘刚

狼疮性肾炎的病理改变,北京大学第一医院肾内科刘刚,重点不在于诊断(但要注意合并其它病变)分型活动性和慢性化指标指导治疗和判断预后,肾脏病理,LN的病理分型,自1974年WHO首次公布LN的病理分型标准后，又分别在1982年、1995年及2003年进行了三次重大修订。,2003 ISN/RPS Consensus Conference on the Classification of Lupus Nephritis (preliminary),I: Minimal mesangial lupus glomerulonephritis (LGN)II: Mesangial proliferative LGNIII: Focal LGN (involving glomeruli, IV-S and IV-G)A/CV: Membranous LGN （可以与III或IV重叠）VI: Advanced sclerotic LGN (90% sclerotic glomeruli),肾脏病理评分,活动指数 慢性指数肾小球病变 1. 细胞增生 1. 肾小球硬化 2. 纤维素样坏死、核碎裂2. 纤维性新月体 3. 细胞性新月体 4. 透明血栓，白金耳 5. 炎细胞浸润肾小管间质病变 1. 单核细胞浸润1. 间质纤维化 2. 肾小管萎缩,Austin et al. 1983,病理报告,狼疮性肾炎IV-G(A/C)V注明特殊病变新月体、纤维素样坏死等肾小管、肾间质、血管等,新分型带来的思考,Class IV: IV-S vs. IV-G?,Comparison of IV-S and IV-G Studies,ParameterNajafi HillYokoyamaMittal et alHTNGSGSGSProteinuriaGSGSGSGSScrGSGSGSGSWire loopsGSGSGSSE depositsGSGSInt InflamGSSGInt FibrosisGSGSSGAIGSGSGSSGCISGGSGSSGOutcome S worseGS (ns)GS (ns)GS (ns),Najafi et al KI 2001, Hill et al KI 2005, Yokohama et al KI 2004, Mittal et al AJKD, 2004,Our work,Renal histopathological data of 327 patients with renal biopsy proven LNDiagnosed between January 2000 and July 2008 in Peking University First Hospital,Lupus. 2009, 18(12):1073-81.,Clinical data,Lab data,Pathological data,Renal Outcome,The frequency of serum ANCA was significantly higher in IV-S group than that in IV-G group (20% vs. 4.6%, P=0.008)The frequencies of anti-C1q IgG1 and IgG3 subclass were significantly higher in IV-G group than that in IV-S group (P=0.006, P=0.011, respectively),CrGN was not rare in patients with LN（33/327） ANCA might play a role in crescent formation.10/33 vs. 3/119 Although aggressive immunosuppressive therapy could achieve clinical remission, their long-term renal outcome was poor.ESRD 7/33 vs. 4/119,Kidney Int. 2009;76:307-317,Kidney International advance online publication, 24 February 2010,5个单位协作313例，有2年以上随访资料,we found that the 2003 ISN/RPS classification system of lupus nephritis, based on glomerular lesions, could also reflect related tubulointerstitial lesions.,141/313肾小球病变重、肾间质小管病变轻15/313肾小球病变轻、肾间质小管病变重,In multivariate Cox hazard analysis of tubulointerstitial lesions, indices of interstitial infiltration, tubular atrophy, and interstitial fibrosis were confirmed as significant independent risk factors for renal outcome.,小结,狼疮性肾炎病理分型尚需根据证据不断修订特殊病变可能具有独特的内在机制和临床特点肾间质小管病变对预后的影响更大,TTP-HUS in LN,Twelve patients with evidence of TMA were identified in 353 patients with LNSeven out of the 12 patients were diagnosed as TTP-HUS,Comparison of clinical and laboratory data between patients with lupus nephritis with and without TTP-HUS,Comparison of renal pathological data between patients with lupus nephritis with and without TTP-HUS,Nephrol Dial Transplant. 2010, 25:145,TTP-HUS was not rare in patients with LNADAMTS-13 autoantibody might play an important role in the pathogenesis of TTP-HUS in LNTheir long-term outcome was poorer compared with “pure” LN,正在进行的研究,研究对象：北京大学第一医院肾内科自2000年1月至2009年6月之间住院确诊的341例LN患者。 入选标准： （1）依据1997年美国风湿病学会制定的SLE分类诊断标准，符合11项中4项或其以上者。 （2）满足 SLE的诊断标准，伴有持续的蛋白尿（0.5g/d或+）和/或管型尿，明确诊断为LN者 。 （3）在我院行肾活检并有完整的临床、病理及随访资料。 （4）肾穿刺标本中肾小球数目10个 ，小动脉数目6个。 Arthritis Rheum.1997,病理评估： （1）病理分型及评分： 按照2003年ISN/RPS制定的LN病理分型标准进行分型； 按照NIH（National Institutes of Health）评分体系进行活动性和慢性化指标评分。 Kidney Int. 2004 Kidney Int. 1984,（2）肾血管病变评分 LN血管病变类型Vascular Immune Complex Deposits (ICD) Noninflammatory Necrotizing Vasculopathy (NNV)Thrombotic Microangiopathy (TMA)True Renal Vasculitis (TRV)Arteriosclerosis (AS) J. Am. Soc. Nephrol. 1994,新AI和CI,血管病变活动性病变评分 ICD (0, 1) NNV (0, 1)