Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
Clin Exp Rheumatol. 2018 Mar-Apr;36 Suppl 111(2):65-72. Epub 2018 Feb 14.
We estimated the cumulative patient survival rates, the causes of death and the initial predictors of death in Korean patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA).
We reviewed the medical records of 153 patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We collected clinical and laboratory data including ANCA, Birmingham vasculitis activity score (BVAS), five factor score (FFS) (2009), comorbidities, medications and prognosis (death and relapse). The hazard ratio (HR) of variables at diagnosis for death in the disease course was assessed by the Cox hazard model analysis.
The mean age of 153 AAV patients (47 men and 106 women) was 55.2 years and the mean follow-up duration was 51.5 months. Fourteen of 153 patients (9.2%) died (7 MPA and 7 GPA patients) during the mean follow-up of 56.9 months. In all patients with AAV, 1 year-, 5 year- and 10 year-cumulative patient survival rates were 96.1%, 94.8% and 92.8%, respectively. The most common cause of death was infection of various causes. FFS (2009) ≥2 (HR 16.520, p=0.012) and diffuse alveolar haemorrhage (DAH) (HR 3.705, p=0.042) at diagnosis could predict death during the follow-up in AAV patients in multivariate COX regression analysis.
The overall mortality rate was 9.2% and 10-year cumulative patient survival rate was 92.8%. At diagnosis, FFS (2009) ≥ 2 and DAH were independent predictors of death during the follow-up in Korean patients with MPA, GPA and EGPA.
我们评估了韩国显微镜下多血管炎（MPA）、肉芽肿性多血管炎（GPA）和嗜酸性粒细胞性 GPA（EGPA）患者的累积患者生存率、死亡原因和死亡的初始预测因素。
我们回顾了 153 例抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）患者的病历。我们收集了包括 ANCA、Birmingham 血管炎活动评分（BVAS）、五因素评分（FFS）（2009 年）、合并症、药物治疗和预后（死亡和复发）在内的临床和实验室数据。通过 Cox 风险模型分析评估了诊断时变量对疾病过程中死亡的风险比（HR）。
153 例 AAV 患者（47 名男性和 106 名女性）的平均年龄为 55.2 岁，平均随访时间为 51.5 个月。在平均 56.9 个月的随访期间，153 例患者中有 14 例（9.2%）死亡（7 例 MPA 和 7 例 GPA 患者）。在所有 AAV 患者中，1 年、5 年和 10 年累积患者生存率分别为 96.1%、94.8%和 92.8%。死亡的最常见原因是各种原因导致的感染。在多变量 COX 回归分析中，诊断时的 FFS（2009 年）≥2（HR 16.520，p=0.012）和弥漫性肺泡出血（DAH）（HR 3.705，p=0.042）是预测 AAV 患者随访期间死亡的独立因素。
总的死亡率为 9.2%，10 年累积患者生存率为 92.8%。在韩国 MPA、GPA 和 EGPA 患者中，诊断时的 FFS（2009 年）≥2 和 DAH 是随访期间死亡的独立预测因素。
