需肾脏替代治疗的ANCA相关肾炎患者的预后及其影响因素

Prognosis and its risk factors in ANCA-associated giomerulonephritis patients treated with initial renal replacement therapy

目的探讨需肾脏替代治疗（RRT）的抗中性粒细胞胞质抗体（ANCA）相关肾炎（AAGN）患者的预后及其影响因素。方法纳入1996年1月至2016年12月南京总医院诊断时即需RRT的AAGN患者100例，男46例，女54例，年龄54（41，60）岁。强化免疫治疗指患者接受激素联合环磷酰胺或吗替麦考酚酯，或联合免疫吸附或血浆置换（IA／DFPP）治疗。分析AAGN患者的肾脏预后及影响因素。结果41例（41％）AAGN患者在1．0（0．5，2．0）个月摆脱RRT（摆脱RRT组），59例（59％）未摆脱RRT转入维持性透析（未摆脱RRT组）。多因素logistic回归分析显示正常肾小球比例〈8％（OR=5．95，P=0．002）、球性硬化比例≥50％（OR=4．87，P=0．003）和未强化免疫治疗（OR=7．81，P=0．004）是AAGN患者不能摆脱透析的影响因素。摆脱RRT的AAGN患者随访22（10，50）个月，15例（36．6％）进入维持性透析，1年和3年的肾存活率分别为86％和60％。100例患者随访6（2，24）个月，共12例（12％）患者死亡，其中4例（4％）为治疗相关。多因素Cox回归分析显示IA／DFPP治疗（HR=10．85，P=0．034）和低白蛋白水平（HR=1．26，P=0．009）是治疗相关死亡的独立影响因素。结论需RRT的AAGN患者摆脱透析比例低，正常肾小球和球性硬化比例及是否接受强化免疫治疗是影响患者摆脱RRT的主要因素，IA／DFPP治疗和低白蛋白水平是治疗相关死亡的独立影响因素。

Objective To explore the prognosis and its risk factors in anti-neutrophil cytoplasmic antibodies （ANCA）-associated glomerulonephritis （AAGN） patients who needed initial renal replacement therapy （RRT）. Methods One hundred patients [ 54 females, 46 males, with a median age of 54 （41, 60） years] with biopsy-proven AAGN and requiring initial RRT between January 1996 and December 2016 in Nanjing Jinling Hospital were included. Intensive immunotherapy indicated that the patients received corticosteroids in combination with cyclophosphamide or mycophenolate mofetil, or immunoadsorption （IA） or double filtration plasmapheresis （DFPP）. The clinical and histological risk factors for renal survival were analyzed. Results Forty-one patients were free of RRT after a median time of 1 （0. 5, 2） month treatment （dialysis-independent group）, and the remaining 59 patients were on maintenance dialysis （dialysis- dependent group）. The multivariate logistic analysis revealed that the proportion of normal glomeruli 〈 8% （OR = 5.95, P = 0. 002） and global sclerotic glomeruli ≥ 50% （ OR = 4. 87, P = 0. 003 ）, and not receiving intensive immunotherapy （ OR = 7. 81, P = 0. 004） were the risk factors for the renal recovery in these patients. During a median follow-up time of 22 （ 10, 50） months, 15 patients（36. 6% ） in the dialysis-independent group progressed into maintenance dialysis, and the 1 and 3 year renal survival rate were 86% and 60%, respectively. During a median follow-up time of 6 （2, 24） months, 12 （12%） patients died, among whom four patients died of therapy. The multivariate Cox regression analysis revealed that IA/DFPP treatment （ HR = 10. 85, P = 0. 034 ） and low albumin level （ HR = 1.26, P = 0. 009 ） significantly associated with a higher risk of therapy-related death. Conclusions The renal recovery rate in AAGN patients with initial RRT was low. The proportion of normal and global sclerotic glomeruli, receiving intensive immunotherapy or not were associated with renal outcome, and IA/DFPP treatment as well as lower albumin level were independently associated with therapy-related death.