Girard T, Mahr A, Noël L H, Cordier J F, Lesavre P, André M H, Guillevin L
Service de Médecine Interne, Hôpital Avicenne, Université Paris-Nord, 125, rue de Stalingrad, 93009 Bobigny Cedex, France.
Rheumatology (Oxford). 2001 Feb;40(2):147-51. doi: 10.1093/rheumatology/40.2.147.
To investigate the predictive value of testing for antineutrophil cytoplasmic antibodies (ANCA) in 55 patients with systemic Wegener's granulomatosis (WG) included in a randomized, prospective trial comparing corticosteroids and oral or pulse cyclophosphamide.
All 55 patients received corticosteroids. A cyclophosphamide pulse of 0.7 g/m2 was given at the time of diagnosis. After the first pulse, the patients were assigned at random to receive either pulse or oral cyclophosphamide (2 mg/kg/day), independently of ANCA results. ANCA were sought using an immunofluorescence assay and an attempt was made to correlate them with relapse of WG. ANCA were monitored throughout the study.
At the time of diagnosis, ANCA were detected in 48 (87%) patients, with a cytoplasmic labelling pattern in 44 and a perinuclear pattern in four. ANCA follow-up was available for 50 patients. ANCA disappeared in 34 patients and persisted in nine. For 79% of the patients, the clinical course improved with the disappearance of ANCA and deteriorated with their persistence or increased titre. Among the patients who were initially ANCA-positive, 23 relapses occurred. Relapses were more frequent when ANCA remained positive or reappeared [13/19 ANCA-positive patients vs 3/29 ANCA-negative patients (P<0.01)]. Nine relapses (39%) occurred in patients with persistent ANCA, and ANCA reappearance preceded relapse in eight (35%). The mean time between inclusion and relapse did not differ between the patients who became ANCA-negative and those who were persistently ANCA-positive (14.6+/-13.2 vs 14.4+/-8.2 months). The mean time to ANCA disappearance was similar for the patients who relapsed and those who did not. Corticosteroids and pulse or oral cyclophosphamide did not significantly modify the time to ANCA disappearance. Throughout the study, seven patients were ANCA-negative.
Although ANCA positivity was associated with relapse, discordance between cytoplasmic ANCA and disease activity was not unusual. In the absence of clinical manifestations, ANCA titres alone can serve as a warning signal but not indicate whether to adjust or initiate treatment.
在一项比较皮质类固醇与口服或脉冲环磷酰胺的随机、前瞻性试验中，研究检测抗中性粒细胞胞浆抗体（ANCA）对55例系统性韦格纳肉芽肿（WG）患者的预测价值。
所有55例患者均接受皮质类固醇治疗。诊断时给予0.7 g/m²的环磷酰胺脉冲治疗。首次脉冲治疗后，患者被随机分配接受脉冲或口服环磷酰胺（2 mg/kg/天），与ANCA结果无关。使用免疫荧光法检测ANCA，并尝试将其与WG的复发相关联。在整个研究过程中对ANCA进行监测。
诊断时，48例（87%）患者检测到ANCA，其中44例为胞浆型，4例为核周型。50例患者有ANCA随访数据。34例患者的ANCA消失，9例持续存在。79%的患者临床病程随ANCA消失而改善，随其持续存在或滴度升高而恶化。在最初ANCA阳性的患者中，发生了23次复发。当ANCA持续阳性或再次出现时，复发更频繁[19例ANCA阳性患者中有13例复发，29例ANCA阴性患者中有3例复发（P<0.01）]。9次复发（39%）发生在ANCA持续存在的患者中，8次复发（35%）中ANCA再次出现先于复发。ANCA转阴的患者与ANCA持续阳性的患者从纳入研究到复发的平均时间无差异（14.6±13.2个月对14.4±8.2个月）。复发患者和未复发患者ANCA消失的平均时间相似。皮质类固醇和脉冲或口服环磷酰胺对ANCA消失的时间无显著影响。在整个研究过程中，7例患者ANCA阴性。
虽然ANCA阳性与复发相关，但胞浆ANCA与疾病活动之间的不一致并不罕见。在无临床表现时，仅ANCA滴度可作为一个警示信号，但不能指示是否调整或开始治疗。
