15 50100 000 12100 000 39100 000 70100

患病率 美国: 15 -50/100, 000 英国: 12/100, 000 瑞典: 39/100, 000 中国(上海): 70/100, 000

Proposal of the International Society of Nephrology and Renal Pathological Society Working Group on the Classification of Lupus Nephritis the major objective is to standardize definitions, emphasize clinically relevant lesions, and encourage uniform and reproducible reporting between centers

2002 ISN/RPS Consensus Conference on the Classification of Lupus Nephritis (preliminary) I: 轻微系膜性狼疮肾炎 II: 系膜增生性狼疮肾炎 III: 局灶性狼疮肾炎 (involving <50% of glomeruli) IV: 弥漫性狼疮肾炎 (involving 50% or > glomeruli, IV-S and IV-G) V: 膜性狼疮肾炎 VI: 严重硬化型狼疮肾炎 (>90% sclerotic glomeruli)

2002 ISN/RPS Consensus Conference on the Classification of Lupus Nephritis (preliminary) 对于 III 、 IV型, 诊断还应该包括: 活动性（A） /活动性和慢性（A/C）/ 慢性（C） 对于 IV型, 诊断还应该包括: 节段性为主 (IV-S) 抑或球性为主 (IV-G) V 型可以同时合并III 型或 IV 型 For classes III and IV, the diagnosis should include the percentage of glomeruli with fibrinoid necrosis and /or cellular crescents when present

Class IV: 弥漫节段 (IV-S) 或 球性 (IV-G) LGN Active or inactive diffuse (50% or more involved glomeruli), endo- and /or extracapillary GN with diffuse subendothelial immune deposits, with or without mesangial alterations. This class is divided into diffuse segmental (IV-S) when >50% of the involved glomeruli have segmental lesions, and diffuse global (IV-G) when >50% of the glomeruli have global lesions IV (A) IV (A/C) IV (C) active diffuse segmental or global proliferative LGN diffuse segmental or global proliferative & sclerotic LGN diffuse segmental or global sclerotic LGN *indicate the proportion of glomeruli with acitve and with sclerotic lesions *indicate the proportion of glomeruli with fibrinoid necrosis and /or crescents

白金耳（wire loop)

CTX静脉或口服? Intermittent pulse therapy has highest therapeutic index; conventional daily CTX (2 mg/kg/d) mostly avoided, or used for ＜ 3 months. 目前国际上应用CTX静脉冲击的方法 l l 评估GFR和体表面积BSA= 身高(cm) x 体重(kg)/3600 初始剂量 0. 75 g/m 2 (如GFR下降1/3, 剂量为 0. 5 g/m 2) 10 -14天检查WBC记数, 保持＞ 1500/ l 逐渐增加CTX剂量至最大 1. 0 g/m 2, 每 3 -4周 1次, 共 6次, 每 3个月1 次, 缓解后共 1年 但CTX静脉用药并没有被广泛接受，仍有很多医生推崇 口服疗法。 Balow JE et al. Lupus Nephritis, In Brady HR, Wilcox C (eds): Therapy in Nephrology and Hypertension. WB Saunders, Philadelphia, pp 130 -137, 1999

SLE复发 1/3 -1/2弥漫增生性狼疮肾炎会复发 抗ds-DNA抗体滴度上升可以预测复发 l l 加强临床随访 CTX预防复发效果肯定，但应考虑副作用 Bootsma H et al. Lancet 345: 1595, 1995 Ciruelo E et al. Arthritis Rheum 39: 2028, 1996 有人建议临床缓解后应用CTX每 3个月静点 1次， 共 1年是目前减少复发最好的方法 Balow JE et al. Lupus Nephritis, In Brady HR, Wilcox C (eds): Therapy in Nephrology and Hypertension. WB Saunders, Philadelphia, pp 130 -137, 1999

狼疮肾炎的预后 5年生存率 All lupus LN Class IV LN 1953 -1969 49% 44% 17% 1970 -1979 82% 67% 55% 1980 -1989 86% 82% 80% 1990 -1995 92% 82% Cameron JS. Lupus Nephritis JASN 1999, 10