摘要
目的 :比较抗中性粒细胞胞浆抗体 (ANCA)阳性 (ANCA +ve)与ANCA阴性 (ANCA -ve)微型多血管炎(MPA)临床与病理的差异 ,探讨ANCA与MPA病情及预后的关系。 方法 :31例微型多血管炎 (MPA) ,其中男 16例 ,女 15例 ,同时采用标准间接免疫荧光 (IF)和ELISA法检测血清ANCA ,根据血清ANCA结果分为ANCA +ve组与ANCA -ve组 ,比较两组肾脏及肾外脏器损害、肾脏病理及预后。两组患者血管炎活动分数 (BVAS1)无差异。 结果 :①血清ANCA :31例MPA中 19例ANCA阳性 (6 1 3% ) ,其中IF ANCA阳性者 8例 ,ELISA法ANCA阳性者 19例。血清抗 GBM抗体均阴性。②发病年龄及病程ANCA +ve组大于ANCA -ve组 ,分别为 (5 6± 11)岁vs(4 8 5± 16 8)岁(P <0 0 5 )和 (30 2± 47 7)月vs (6 5± 8 8)月 (P <0 0 1)。ANCA +ve组女性多于男性 (男 /女 8/ 11) ,而ANCA -ve组男性多见 (男 /女 8/ 4)。③ANCA +ve组需透析病例数及活检时SCr水平 (5 88± 334 ) μmol/L高于ANCA -ve(4 87±2 84) μmol/L(P <0 0 5 ) ,贫血程度更为严重 ,Hb(78 9± 16 2 )g/Lvs (95 5± 2 2 0 )g/L(P <0 0 1)。肾活检显示ANCA+ve组新月体比例显著高于ANCA -ve组 (6 1 7± 33 2 ) %vs (33 4± 32 8) % (P <0 0 1) ,ANCA -ve组细胞性新月体所占比例?
Objective:The incidence of ANCA negative MPA was reported to be higher in Chinese patients.In this study,we compared the differences of clinical and pathological characteristics between ANCA positive and ANCA negative MPA. Methodology:Thirty one Chinese patients(16 males and 15 females)with clinical and renal biopsy proved MPA were included in the study.ANCAs were detected with both standard indirect immanofluorescence(IIF)assay and ELISA for MPO ANCA or PR3 ANCA.The patients were divided into ANCA positive(ANCA+ve)and ANCA negative(ANCA-ve)groups.Serum anti GBM antibody and ANA were also detected with IIF assay.The activity of disease was measured with Birminham vasculitis activity score(BVAS1)system. Results:ANCA was positive in 19 of 31 patients(61 3%),among which 58 1% were MPO ANCA,3 2% were PR3-ANCA.Anti GBM antibody and ANA were all negative in both groups.No immunosuppressive drug were given to the patients in ANCA-ve group prior to the admission.BVAS1 scores were compatible in ANCA+ve and ANCA-ve group.Comparing with ANCA-ve,the cases of ANCA+ve manifested with older age(56±11y vs 48 5±16 5y, P <0 05),longer duration of disease (30 2±47 7m vs 6 5±8 8m)and more?females?(8M/11F vs 8M/4F).?Clinical?manifestations?showed?differences?inANCA+ve vs ANCA-ve:the rate of case with RPGN 42 1% vs 58 3%;with CRF and ESRF,57 9% vs 41 6%;renal enlargement,47 4% vs 75%;gross hematuria,21 7% vs 50%;serum creatinine,588±334μmol/L vs 487±284 μmol/L( P <0 05);Hb,78 9±16 2g/L vs 95 5±22 0g/L( P <0 05).Renal biopsy revealed more crescent formation in ANCA+ve than in ANCA-ve group(61 7±33 2% vs 33 4±32 8%, P <0 01).Extra renal involvement in ANCA+ve vs ANCA-ve was investigated:the cases with more than two extra renal involvements were 63 2% vs 33 3%;haemoptysis,42 1% vs 0;diffuse pulmonary damage,15 8% vs 0;skin invdvement,15 8% vs 41 7%;GI,eye,joints,nervous system and cardic involvement were compatible in two groups.The treatment regimens with steroids and cyclophospahmide or mycophenolate mofetil had no difference in two groups.We noticed that the ANCA+ve showed worse prognosis than that of ANCA-ve,the rate of SCr returning to normal was 5 3% vs 33 3% respectively;the mean SCr after treatment was 223 8±126 0 vs 159 3±66 2 μmol/L in nondialysis patients;proporation of maintenance dialysis,21 1% vs 25%.Death rate was 26 3% in ANCA+ve group while;none in ANCA ve group. Conclusion:This study showed that patients with ANCA+ve and ANCA-ve MPA had some differences in clinical manifestations,renal histological lesion and prognosis.Further studies are needed to explore the different pathogenesis and to investigate how the ANCA influencing the efficacy of the current therapy.
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
2001年第3期205-210,共6页
Chinese Journal of Nephrology,Dialysis & Transplantation
