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系统性红斑狼疮中的抗中性粒细胞胞浆抗体(ANCA):患病率、临床关联及与其他自身抗体的相关性

Anti-neutrophil cytoplasmic antibodies (ANCA) in systemic lupus erythematosus: prevalence, clinical associations and correlation with other autoantibodies.

作者信息

Pradhan Vandana D, Badakere S S, Bichile Lata S, Almeida A F

机构信息

Department of Autoimmune disorders, Institute of Immunohaematology, Indian Council of Medical Research, 13th floor, KEM Hospital, Mumbai 400 012.

出版信息

J Assoc Physicians India. 2004 Jul;52:533-7.

Abstract

AIM

This study was undertaken to clarify the nature of anti-neutrophil cytoplasmic antibodies (ANCA) along with other autoantibodies in lupus nephritis (LN) patients and in systemic lupus erythematosus (SLE) patients without nephritis and to know their correlation with clinical manifestations and presence of other autoantibodies.

MATERIAL AND METHODS

Fourty one LN patients and 18 SLE patients without nephritis were studied. LN patients were subdivided into diffuse proliferative glomerulonephritis (DPGN), focal proliferative glomerulonephritis (FPGN), rapidly progressive glomerulonephritis (RPGN) and membranoproliferative glomerulonephritis (MPGN). Anti-neutrophil cytoplasmic antibodies (ANCA) were detected by indirect immunofluorescence and confocal laser scanning microscope using PMN and HL60 cells. ANCA specificities like anti-myeloperoxidase (anti-MPO), anti-proteinase 3 (anti-PR3), anti-lactoferrin (anti-LF) and anti-cathepsin G (anti-CG) were detected by ELISA. Other autoantibodies like anti-nuclear antibodies (ANA), anti-double stranded DNA (anti-dsDNA), anti-single stranded DNA(anti-ssDNA), anti-ribonucleoproteins (anti-nRNP), anti-Smith antibodies (anti-Sm) and rheumatoid factor (RF) were also tested.

RESULTS

ANCA was detected in 37.3% patients. The predominant ANCA pattern was perinuclear (p-ANCA). ANCA positivity was higher in LN patients and when confirmed by ELISA, 54.5% ANCA positives had anti-myeloperoxidase (anti-MPO). The cytoplasmic ANCA (c-ANCA) pattern was not seen in any patient. Two patients having FPGN with crescents showed atypical 'X-ANCA' pattern with dual specificity to anti-MPO and anti-PR3 by ELISA. The titers of ANCA were more in LN as compared to SLE without nephritis. LN cases having DPGN, FPGN, RPGN with crescents had higher titer p-ANCA positivity with corresponding anti-MPO antibodies, along with ANA, anti-dsDNA, anti-ssDNA and anti-Sm + anti-nRNP and also high SLEDAI scores.

CONCLUSION

ANCA in SLE may be used as a serological marker along with clinical and histopathological assessment to differentiate vasculitides in LN cases from SLE without nephritis.

摘要

目的

本研究旨在阐明狼疮性肾炎(LN)患者以及无肾炎的系统性红斑狼疮(SLE)患者中抗中性粒细胞胞浆抗体(ANCA)及其他自身抗体的性质,并了解它们与临床表现及其他自身抗体存在情况的相关性。

材料与方法

对41例LN患者和18例无肾炎的SLE患者进行研究。LN患者被细分为弥漫性增殖性肾小球肾炎(DPGN)、局灶性增殖性肾小球肾炎(FPGN)、快速进展性肾小球肾炎(RPGN)和膜增殖性肾小球肾炎(MPGN)。使用PMN和HL60细胞,通过间接免疫荧光和共聚焦激光扫描显微镜检测抗中性粒细胞胞浆抗体(ANCA)。通过酶联免疫吸附测定(ELISA)检测ANCA特异性,如抗髓过氧化物酶(抗MPO)、抗蛋白酶3(抗PR3)、抗乳铁蛋白(抗LF)和抗组织蛋白酶G(抗CG)。还检测了其他自身抗体,如抗核抗体(ANA)、抗双链DNA(抗dsDNA)、抗单链DNA(抗ssDNA)、抗核糖核蛋白(抗nRNP)、抗史密斯抗体(抗Sm)和类风湿因子(RF)。

结果

37.3%的患者检测到ANCA。主要的ANCA模式是核周型(p-ANCA)。LN患者中ANCA阳性率更高,经ELISA确认后,54.5%的ANCA阳性患者具有抗髓过氧化物酶(抗MPO)。任何患者均未出现胞浆ANCA(c-ANCA)模式。两名患有伴有新月体的FPGN患者通过ELISA显示出对抗MPO和抗PR3具有双重特异性的非典型“X-ANCA”模式。与无肾炎的SLE相比,LN患者中ANCA的滴度更高。患有DPGN、FPGN、伴有新月体的RPGN的LN病例具有更高滴度的p-ANCA阳性以及相应的抗MPO抗体,同时伴有ANA、抗dsDNA、抗ssDNA和抗Sm +抗nRNP,且SLE疾病活动指数(SLEDAI)评分也较高。

结论

在SLE中,ANCA可作为一种血清学标志物,结合临床和组织病理学评估,以区分LN病例中的血管炎与无肾炎的SLE。

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