目的:分析抗中性粒细胞胞质抗体(ANCA)相关性血管炎(AAV)患者的临床及肾脏病理特征,探讨伴补体沉积AAV的临床特点. 方法:选取2006年1月至2012年1月南京医科大学第一附属医院临床诊断为AAV的患者96例,回顾性分析其临床及肾脏病理特征. 结果:(1)96例AAV患者中男53例、女43例,年龄15～ 83岁,平均起病年龄58.4±16.6岁,其中42.3％的患者年龄≥65岁；病程30～ 115d(中位病程57d).(2)肾脏受累84例(87.5％),肺部受累61例(63.5％).(3)51例肾活检患者中,86.3％患者有肾小球新月体形成,54.9％伴毛细血管袢纤维素样坏死,64.7％伴球性硬化,64.7％有肾小管间质纤维化.44例病理诊断为AAV肾损害,根据其光镜下肾小球病理改变的程度将其分类为局灶型(15例)、新月体型(7例)、混合型(12例)及硬化型(10例).局灶型的患者发病时的血清肌酐(SCr)水平明显低于新月体型、混合型和硬化型(P＜0.01、P＜0.05、P＜0.05)；免疫抑制治疗后,新月体型AAV患者SCr下降幅度显著大于局灶型、混合型及硬化型(P＜0.01、P＜0.01、P＜0.05).随访中,上述四型分别有1例、2例、2例、3例进入终末期肾病(ESRD).(4)伴补体沉积阳性组(n=22)与补体沉积阴性组(n=17)相比,前者发病时血沉明显增快(P =0.023),估算肾小球滤过率、血清补体C3水平明显降低(P =0.033、P=0.005),SCr和尿素氮明显升高(P =0.021、P=0.012),肾脏病理中纤维性新月体和球性硬化比例明显升高(P=0.009、P=0.028),而正常肾小球比例减少(P =0.016). 结论:本组AAV患者临床表现以肾脏及肺部损害最常见.局灶型患者肾功能损害相对较轻,新月体型患者对免疫抑制治疗反应较好,硬化型患者进展为ESRD的比例高于另外三型患者,提示预后不佳；AAV患者肾脏病理中出现补体沉积并不少见,伴补体沉积者肾脏损害较重,应引起重视.%Objective: To investigate the clinical and renal pathological characteristics of patients with ANCA associated vasculitis ( AAV) , and to explore the clinical feature of patients with complement deposition in renal pathology. Methodology:From Jan,2006 to Jan,2012,ninety six patients diagnosed as AAV were collected and their clinical and pathological data were retrospectively analyzed. Results: They were 53 males and 43 females ( ratio 1.2: 1) with an age 58. 4 ± 16. 6 (ranged from 15 ~83) years old,and older than 65 years old in 42. 3% . The median duration of disease was 57 days from 30 to 115 days. Their clinical manifestations were diverse and complicated. The most commonly involved organs were kidney in 84 cases (87. 5% ) ,and lung in 61 (63. 5% ). In renal pathology;51 of 96 patients were performed renalrnbiopsy. The histological examinations showed crescent formation in 86. 3% ,fibrinoid necrosis of glomeruli in 54. 9% ,global sclerosis in 64. 7% and tubular interstitial fibrosis in 62. 7%. According to the degree of glomerular pathological change assessed by light microscopy ,44 cases with pathological diagnosis of AAV were classified as four general categories: Focal (15) , Crescentic (7) , Mixed (12) and Sclerotic (10 cases). The patients of focal subtype presented with significantly lower serum creatinine than that the other three subtypes ( Pd2 < 0. 01、Pb1 < 0. 05、and Pc1 < 0. 05 ). After the immunosuppressive therapy, the patients of crescentic subtype had a bigger decrease of serum creatine, compared with those who presented with other subtypes ( Pa2 < 0. 01、Pb2 < 0. 01、Pc1< 0. 05 ) . ESRD developed in one of 15 patients with focal, 2 of 7 patients with crescentic,2 of 12 patients with mixed,and 3 of 10 patients with sclerotic subtype vasculitis during the follow-up period. The patients with complement deposition ( n = 22 ) had a higher level of erythrocyte sedimentation rate ( ESR) ( P = 0. 023 ) , higher initial serum creatinine ( P = 0. 021 ) and serum ureanitrogen ( P = 0. 012 ) , lower eGFR ( P = 0. 033 ) and serum C3 ( P = 0. 005) at presentation, compared with those without complement deposition ( n = 17 ) . In renal histology, the patients with complement deposition had a notably higher percentage in fibrous crescents ( P = 0. 009 ) and global sclerosis ( P = 0. 028 ) but a lower percentage of normal glomeruli (P =0. 016). Conclusion; The most common involved organs were kidney and lung in our patients. The patients with focal presented with relatively preserved renal function; with crescentic responded well to treatment; and with sclerotic subtype had the highest percentage of developing ESRD and a poor prognosis. The complement deposition in glomeruli was not rare in patients with ANCA-associated glomerulonephritis, which was associated with more severe renal injury.
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