韦格纳肉芽肿病患者的损伤和生存预测因素：50 例患者分析。

Predictors of damage and survival in patients with Wegener's granulomatosis: analysis of 50 patients.

机构信息

Division of Rheumatology, Department of Internal Medicine, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.

出版信息

J Rheumatol. 2010 Feb;37(2):374-8. doi: 10.3899/jrheum.090387. Epub 2009 Dec 15.

DOI:10.3899/jrheum.090387

PMID:20008921

Abstract

OBJECTIVE

To evaluate damage features and impact on survival by Vasculitis Damage Index (VDI) in a cohort of Turkish patients with Wegener's granulomatosis (WG).

METHODS

We enrolled 50 (25 female) patients with WG according to ACR criteria. Birmingham Vasculitis Activity Score (BVAS) and VDI were used to analyze disease activity and damage.

RESULTS

Patients had kidney (82%), upper airway (72%), lung (70%), and nervous system (15%) involvement. Median age at diagnosis was 45 years, time to diagnosis was 3.5 months, and total followup time was 35.5 months. All but one patient was positive for antineutrophil cytoplasmic antibodies (ANCA). Mean final dose and duration of corticosteroid and cyclophosphamide was 15 +/- 14 g, 39 +/- 33 months and 36 +/- 34 g, 21 +/- 2 months, respectively. Mean early (e) BVAS were 20.2 +/- 7.1 (4-38) (median 21). Mean e-BVAS and e-VDI scores at presentation and final (f)-VDI scores at last visit were 20.2 +/- 7.1 (4-38), 3.1 +/- 1.7 (median 3) (0-7) and 4.4 +/- 2.6 (0-12), consecutively. Disease related damage was prominent in kidneys (50%) and upper airways (27%). Amenorrhea (90%), cataract (28%), and diabetes (24%) were the most frequent treatment related damages. Rapidly progressive glomerulonephritis at presentation (42%) progressed to endstage renal failure in 20%. Relapses occurred in 25% with mean BVAS of 6.5 +/- 2.3 (4-11). Survival rate was 77% at 37 months. Deaths occurred early (90% in the first year). f-VDI was high in patients who relapsed (6 +/- 3 vs 3.8 +/- 2.1, p = 0.03). Logistic regression analysis demonstrated that age at time of diagnosis and e-VDI were lower in survivors with OR = 0.9 (p = 0.06, 95% CI: 0.78-1) and OR = 0.5 (p = 0.04, 95%CI: 0.25-0.98), respectively. In this cohort, e-VDI score of 5 or more was related to death with 98% sensitivity and 56% specificity (p = 0.004) (CI: 0.66-0.95).

CONCLUSION

Disease related damage outweighed treatment related damage in our cohort of predominantly generalized disease activity. Early damage and older age were found to be predictors of final damage and death.

摘要

目的

通过血管炎损伤指数（VDI）评估土耳其韦格纳肉芽肿（WG）患者队列中的损伤特征及其对生存的影响。

方法

我们根据 ACR 标准纳入了 50 名（25 名女性）WG 患者。采用伯明翰血管炎活动评分（BVAS）和 VDI 分析疾病活动度和损伤。

结果

患者有肾脏（82%）、上呼吸道（72%）、肺部（70%）和神经系统（15%）受累。诊断时的中位年龄为 45 岁，诊断时间为 3.5 个月，总随访时间为 35.5 个月。除 1 例患者外，所有患者的抗中性粒细胞胞质抗体（ANCA）均为阳性。皮质类固醇和环磷酰胺的最终剂量和持续时间分别为 15 ± 14 g、39 ± 33 个月和 36 ± 34 g、21 ± 2 个月。早期（e）BVAS 的平均（均数 ± 标准差）为 20.2 ± 7.1（4-38）（中位数 21）。就诊时的平均 e-BVAS 和 e-VDI 评分以及末次就诊时的终末（f）-VDI 评分分别为 20.2 ± 7.1（4-38）、3.1 ± 1.7（中位数 3）（0-7）和 4.4 ± 2.6（0-12）。肾脏（50%）和上呼吸道（27%）的疾病相关损伤最为突出。闭经（90%）、白内障（28%）和糖尿病（24%）是最常见的治疗相关损伤。就诊时快速进展性肾小球肾炎（42%）中有 20%进展为终末期肾衰竭。25%的患者发生复发，平均 BVAS 为 6.5 ± 2.3（4-11）。37 个月时的生存率为 77%。死亡发生较早（90%在第一年）。复发患者的 f-VDI 较高（6 ± 3 与 3.8 ± 2.1，p = 0.03）。逻辑回归分析表明，诊断时的年龄和 e-VDI 是生存者的较低，OR = 0.9（p = 0.06，95%CI：0.78-1）和 OR = 0.5（p = 0.04，95%CI：0.25-0.98）。在本队列中，e-VDI 评分≥5 与死亡相关，敏感性为 98%，特异性为 56%（p = 0.004）（CI：0.66-0.95）。