Department of Medicine, Kidney Center, Tokyo Women's Medical University, Tokyo, Japan.
Nephron Clin Pract. 2010;115(1):c21-7. doi: 10.1159/000286346. Epub 2010 Feb 19.
BACKGROUND/AIMS: We conducted a broad survey of 99 patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and investigated both prognosis and outcomes.
Clinical data evaluated were age, sex, patient survival, renal survival, serum albumin, serum creatinine, urinary protein, hematuria, C-reactive protein (CRP), ANCA titer, IgG and the Birmingham Vasculitis Activity Score (BVAS).
The patient survival rate at 6 months after onset was 84.8%, and that at 2 years after onset was 82.0%. Most deaths were within 6 months of onset. Infection accounted for 9 deaths (60.0%). Infection together with pulmonary involvement of active vasculitis accounted for 2 deaths (13.3%). Organ-specific involvement of active vasculitis alone caused 3 deaths (20.0%). Others died of cardiac events. At 1 and 3 months after onset, BVAS (p < 0.0001, p = 0.002), albumin (p = 0.006, p = 0.0004) and CRP (p = 0.04, p = 0.0002) were also associated with patient death.
To improve the prognosis of those with ANCA-associated vasculitis, the intensity of initial treatment should be aimed at disease severity. Employing BVAS improved the ability to evaluate therapeutic responses. Finally, prescription with sulfamethoxazole-trimethoprim during the induction therapy with immunosuppressive agents may be advised.
背景/目的：我们对 99 例抗中性粒细胞胞浆抗体（ANCA）相关性血管炎患者进行了广泛调查，同时调查了预后和结局。
评估的临床数据包括年龄、性别、患者生存、肾脏生存、血清白蛋白、血清肌酐、尿蛋白、血尿、C 反应蛋白（CRP）、ANCA 滴度、IgG 和伯明翰血管炎活动评分（BVAS）。
发病后 6 个月的患者生存率为 84.8%，发病后 2 年的生存率为 82.0%。大多数死亡发生在发病后 6 个月内。感染导致 9 例死亡（60.0%）。感染合并活动性血管炎的肺部受累导致 2 例死亡（13.3%）。单纯的活动性血管炎的器官特异性受累导致 3 例死亡（20.0%）。其他患者死于心脏事件。发病后 1 个月和 3 个月时，BVAS（p<0.0001，p=0.002）、白蛋白（p=0.006，p=0.0004）和 CRP（p=0.04，p=0.0002）也与患者死亡相关。
为了改善 ANCA 相关性血管炎患者的预后，初始治疗的强度应针对疾病严重程度。采用 BVAS 可提高评估治疗反应的能力。最后，建议在免疫抑制剂诱导治疗期间使用磺胺甲恶唑-甲氧苄啶进行处方。
