抗中性粒细胞胞质抗体相关性间质性肺疾病急性加重的临床意义：不良预后的指标。
Clinical significance of acute exacerbation in interstitial lung disease with antineutrophil cytoplasmic antibody: an indicator of poor prognosis.
机构信息
Department of Respiratory, The Affiliated Hospital of Medical School of Ningbo University, Ningbo, China.
Department of Respiratory, The Affiliated Hospital of Medical School of Ningbo University, Jiangbei District, Ningbo City People's Road No. 247, Ningbo, Zhejiang 315020, China.
出版信息
Ther Adv Respir Dis. 2022 Jan-Dec;16:17534666221140974. doi: 10.1177/17534666221140974.
BACKGROUND
The association between interstitial lung disease (ILD) and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has been increasingly recognized in recent years. The clinical features and prognostic differences between AAV-associated ILD and isolated ANCA-positive idiopathic interstitial pneumonias (IIPs) remain unclear. The purpose of this study was to determine the clinical significance and prognosis of ANCA-positive ILD to further guide clinical management.
METHODS
This study retrospectively reviewed the data of 379 ILD patients with available ANCA results and ultimately analysed 49 ANCA-positive patients. AAV diagnosis was based on the 2012 revised Chapel Hill Consensus Conference (CHCC) criteria, and 33 of 49 patients were diagnosed with microscopic polyangiitis (MPA). The baseline clinical information and laboratory parameters were collected and analysed at each patient's initial diagnosis.
RESULTS
Among 49 ANCA-positive ILD patients, the high-resolution computed tomography (HRCT) pattern was mainly usual interstitial pneumonia (UIP) (59.18%), followed by nonspecific interstitial pneumonia (NSIP) (26.53%). The C-reactive protein (CRP) level (43.89± 40.61 18.74± 20.05, = 0.028) and erythrocyte sedimentation rate (ESR) (71.97± 42.73 versus 40.69± 28.46, = 0.011) were significantly higher in the MPA-ILD group than in the ANCA-IIP group. Haemoglobin (113.09 ± 24.47 132.19± 13.34, = 0.006) and albumin (32.95± 5.84 36.52± 3.94, = 0.032) levels were significantly lower. Survival was shorter among MPA-ILD patients than among ANCA-IIP patients [hazard ratio (HR) 3.38, 95% confidence interval (CI) 1.32-8.67, = 0.040]. In the multivariable Cox analysis, a diagnosis of MPA (HR 3.91, 95% CI 1.07-14.08, = 0.038) and acute exacerbation (AE) of ILD (HR 9.43, 95% CI 2.89-30.30, < 0.001) were significantly independently associated with shorter survival in ANCA-positive ILD patients, and the NSIP pattern (HR 0.07, 95% CI 0.01-0.41, = 0.003) was independently associated with prolonged survival.
CONCLUSION
ANCA-ILD patients mostly have myeloperoxidase (MPO)-ANCA positivity and an MPA diagnosis. Survival was shorter among MPA-ILD patients than among ANCA-IIP patients. Respiratory failure and AE were associated with poorer prognosis. Early antifibrotic treatment may be a reasonable treatment option in fibrotic ILD patients with ANCA positivity.
背景
近年来，间质性肺病（ILD）与抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）之间的关联已得到越来越多的认识。AAV 相关性 ILD 与单纯 ANCA 阳性特发性间质性肺炎（IIP）之间的临床特征和预后差异仍不清楚。本研究旨在确定 ANCA 阳性 ILD 的临床意义和预后，以进一步指导临床管理。
方法
本研究回顾性分析了 379 例有可用 ANCA 结果的 ILD 患者的数据，最终分析了 49 例 ANCA 阳性患者。AAV 的诊断依据是 2012 年修订的查普希尔共识会议（CHCC）标准，其中 33 例患者被诊断为显微镜下多血管炎（MPA）。在每位患者的初始诊断时收集并分析了基线临床信息和实验室参数。
结果
在 49 例 ANCA 阳性 ILD 患者中，高分辨率计算机断层扫描（HRCT）模式主要为普通间质性肺炎（UIP）（59.18%），其次是非特异性间质性肺炎（NSIP）（26.53%）。MPA-ILD 组的 C 反应蛋白（CRP）水平（43.89±40.61 18.74±20.05，=0.028）和红细胞沉降率（ESR）（71.97±42.73 对比 40.69±28.46，=0.011）显著高于 ANCA-IIP 组。血红蛋白（113.09±24.47 132.19±13.34，=0.006）和白蛋白（32.95±5.84 36.52±3.94，=0.032）水平显著较低。MPA-ILD 患者的生存时间短于 ANCA-IIP 患者[风险比（HR）3.38，95%置信区间（CI）1.32-8.67，=0.040]。在多变量 Cox 分析中，MPA 诊断（HR 3.91，95%CI 1.07-14.08，=0.038）和 ILD 急性加重（AE）（HR 9.43，95%CI 2.89-30.30，=0.001）与 ANCA 阳性 ILD 患者的生存时间缩短显著相关，而 NSIP 模式（HR 0.07，95%CI 0.01-0.41，=0.003）与生存时间延长显著相关。
结论
ANCA-ILD 患者大多为髓过氧化物酶（MPO）-ANCA 阳性和 MPA 诊断。MPA-ILD 患者的生存时间短于 ANCA-IIP 患者。呼吸衰竭和 AE 与预后不良相关。早期抗纤维化治疗可能是 ANCA 阳性纤维化 ILD 患者的合理治疗选择。
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