Slot Marjan C, Tervaert Jan Willem Cohen, Boomsma Maarten M, Stegeman Coen A
University Hospital Maastricht, Maastricht, The Netherlands.
Arthritis Rheum. 2004 Apr 15;51(2):269-73. doi: 10.1002/art.20234.
To analyze disease-free survival in patients with antineutrophil cytoplasmic antibody (ANCA)-associated small-vessel vasculitis (AAV) treated with cyclophosphamide only or switched to azathioprine after 3 months of full remission while taking cyclophosphamide.
We analyzed disease-free survival in all consecutive patients diagnosed with AAV between 1990 and 2000 at our center. Patients were treated with cyclophosphamide only (1990-1996) or switched to azathioprine after 3 months of remission while taking cyclophosphamide (1997-2000). All patients received at least 12 months of followup.
Of the total 128 patients, 53 (41%) relapsed. Forty-four of the 128 patients (34%) had been switched to azathioprine therapy. Disease-free survival at 2 and 4 years was 76% and 65% in the cyclophosphamide group compared with 76% and 51% in the azathioprine group. In patients with proteinase 3 (PR3) classic ANCA (C-ANCA)-associated vasculitis who were switched to azathioprine (n = 33), a positive C-ANCA titer at the moment of treatment switch (n = 13) was significantly associated with relapse (RR 2.6, 95% confidence interval 1.1-8.0; P = 0.04). In patients with a negative ANCA titer at the time of switch to azathioprine, disease-free survival at 2 and 4 years was 80% and 62%, which was identical to that for patients treated with cyclophosphamide only. In patients who were ANCA-positive at the time of treatment switch, disease-free survival at 2 and 4 years was only 58% and 17%.
Switching cyclophosphamide to azathioprine after induction of remission in patients with PR3-ANCA-associated vasculitis who are still ANCA-positive at the time of treatment switch is associated with a high risk of relapse.
分析仅接受环磷酰胺治疗或在环磷酰胺治疗3个月完全缓解后改用硫唑嘌呤治疗的抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎（AAV）患者的无病生存期。
我们分析了1990年至2000年在我们中心连续诊断为AAV的所有患者的无病生存期。患者仅接受环磷酰胺治疗（1990 - 1996年）或在环磷酰胺治疗缓解3个月后改用硫唑嘌呤治疗（1997 - 2000年）。所有患者均接受了至少12个月的随访。
128例患者中，53例（41%）复发。128例患者中有44例（34%）改用了硫唑嘌呤治疗。环磷酰胺组2年和4年的无病生存率分别为76%和65%，硫唑嘌呤组分别为76%和51%。在改用硫唑嘌呤的蛋白酶3（PR3）经典ANCA（C - ANCA）相关性血管炎患者（n = 33）中，治疗转换时C - ANCA滴度阳性（n = 13）与复发显著相关（相对危险度2.6，95%置信区间1.1 - 8.0；P = 0.04）。在改用硫唑嘌呤时ANCA滴度阴性的患者中，2年和4年的无病生存率分别为80%和62%，与仅接受环磷酰胺治疗的患者相同。在治疗转换时ANCA阳性的患者中，2年和4年的无病生存率仅为58%和l7%。
对于PR3 - ANCA相关性血管炎患者，在诱导缓解后改用硫唑嘌呤治疗，若治疗转换时仍ANCA阳性，则复发风险高。
