显微镜下多血管炎肺受累患者的临床特征及预后分析

Clinical features and prognosis of microscopic polyangiitis with pulmonary involvement

摘要:

目的 探究显微镜下多血管炎（microscopic polyangiitis，MPA）肺受累的临床特征与预后因素分析。方法 回顾性分析2014年1月1日至2021年3月1日于重庆医科大学附属第一医院确诊及随访至2022年3月1日的237例MPA肺受累患者临床资料。采用Kaplan-Meier方法和Cox比例风险回归模型分析生存数据，得出累积生存率及预后因素。结果 MPA肺受累患者的平均年龄是65.18岁，中位随访时间为26.13个月。在纳入研究的237例患者中，MPA伴弥漫性肺泡出血（diffuse alveolar haemorrhage，DAH）患者98例（41.35%）。MPA肺受累患者1年、3年、5年累积生存率分别为67.50%、54.10%、43.00%。中位生存时间为45.03（0.10，97.73）个月。MPA伴DAH患者1年、3年、5年累积生存率分别为63.30%、49.40%、40.20%。Cox多因素回归分析显示年龄>65岁（HR=1.981，95%CI=1.244~3.154，P=0.004）、白细胞总数（white blood cell，WBC）>10×10⁹个/L（HR=1.860，95%CI=1.170~2.956，P=0.009）、血清肌酐（serum creatinine，sCr）>250 μmol/L（HR=1.987，95%CI=1.217~3.243，P=0.006）、氧合指数<300 mmHg（HR=2.780，95%CI=1.696~4.557，P<0.001）、诊断时第三版伯明翰血管炎活动性评分（Birmingham vasculitis activity score version 3，BVASv.3）（HR=1.040，95%CI=1.006~1.075，P=0.020）是影响患者生存时间的独立预测因素（P<0.05）。结论 MPA肺受累患者生存率低下，年龄>65岁、WBC>10×10⁹个/L、sCr>250 μmol/L、氧合指数<300 mmHg、诊断时BVASv.3是MPA肺受累预后不良的独立危险因素。

Abstract:

Objective To investigate the clinical features and prognostic factors of microscopic polyangiitis（MPA） with pulmonary involvement.Methods A retrospective analysis was performed for the clinical data of 237 MPA patients with pulmonary involvement who were diagnosed and followed up till March 1，2021 in The First Affiliated Hospital of Chongqing Medical University from January 1， 2014 to March 1， 2021. The Kaplan-Meier method and the Cox proportional-hazards regression model were used to analyze survival data and obtain cumulative survival rate and prognostic factors.Results The MPA patients with pulmonary involvement had a mean age of 65.18 years and a median follow-up time of 26.13 months. Among the 237 patients enrolled in this study，98（41.35%） had MPA with diffuse alveolar hemorrhage（DAH）. The 1-，3-，and 5-year cumulative survival rates were 67.50%， 54.10%， and 43.00%，respectively，for the MPA patients with pulmonary involvement， with a median survival time of 45.03 （0.10， 97.73） months. The 1-， 3-，and 5-year cumulative survival rates were 63.30%，49.40%，and 40.20%，respectively，for the patients with MPA with DAH. The multivariate Cox regression analysis showed that age>65 years［hazard ratio（HR）=1.981，95%CI=1.244-3.154，P=0.004］，white blood cell count（WBC）>10×10⁹/L（HR=1.860，95%CI=1.170-2.956，P=0.009），serum creatinine（SCr）>250 μmol/L（HR=1.987，95%CI=1.217-3.243，P=0.006），PaO₂/FiO₂<300 mmHg（HR=2.780，95%CI=1.696-4.557，P<0.001），and Birmingham vasculitis activity score version 3（BVASv.3） at the time of diagnosis（HR=1.040，95%CI=1.006-1.075，P=0.020） were independent influencing factors for survival time（P<0.05）.Conclusion MPA patients with pulmonary involvement tend to have a low survival rate. Age>65 years，WBC>10×10⁹/L，SCr>250 μmol/L， PaO₂/FiO₂<300 mmHg， and BVASv.3 at the time of diagnosis are independent risk factors for the poor prognosis of MPA with pulmonary involvement.