Pulmonary fibrosis in myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitides
Pulmonary fibrosis in myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitides
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DOI：
10.1111/j.1440-1843.2004.00581.x
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发表时间：
2004-06-01
期刊：
影响因子：
6.9
通讯作者：
Nakata, K
中科院分区：
文献类型：
--
作者：
Homma, S;Matsushita, H;Nakata, K
目的：肺纤维化（PF）与髓过氧化物酶抗中性粒细胞胞浆抗体（MPO-ANCA）相关血管炎的关系尚未得到充分证实。本研究的目的是评估 MPO-ANCA 检测呈阳性的患者 PF 的临床病理学特征。方法：在本研究中，对 31 名被诊断为 PF 的患者（17 名男性和 14 名女性；平均年龄，69 岁）进行了临床病理学评估，其 MPO-ANCA 水平呈阳性，范围为 10 至 840 EU，平均为 112.5 EU。结果：在 31 名 PE 患者中，22患有胶原血管疾病等潜在全身性疾病，另有 9 人病因不明。 14 名患者显示出肾小球肾炎的证据。所有 31 名患者的临床特征均为干咳和/或细湿啰音病史。胸部 CT 扫描显示 26 名患者的肺底呈蜂窝状。所有 11 例尸检病例中病变肺组织的组织病理学特征均符合常见的间质性肺炎 (UIP) 模式。 5 名患者的支气管动脉和/或肺小动脉确诊为血管炎。 31名患者中，死亡人数高达13人。死亡原因为：5 例肺功能恶化（其中 2 例与肺出血有关）、2 例肺癌、4 例肺炎、2 例消化道出血。比较了 MPO-ANCA 阴性胶原血管疾病、隐源性纤维化肺泡炎 (CIA) 和 MPO-ANCA 阳性 PF 的存活率。 MPO-ANCA 阳性 PF 的 5 年生存率比 MPO-ANCA 阴性胶原血管疾病的 PF 差，与 CIA 相同。 结论：尽管 MPO-ANCA 滴度与 PE 活性之间没有相关性，但本研究表明，MPO-ANCA 阳性的存在是 PE 患者的不利预后因素。
Objective: The association of pulmonary fibrosis (PF) with myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitides has not been well documented. The aim of this study was to assess the clinicopathological characteristics of PF in patients who tested positive for MPO-ANCA.Methodology: In this study, 31 patients (17 males and 14 females; mean age, 69 years) diagnosed as having PF with positive MPO-ANCA levels ranging from 10 to 840 EU with a mean of 112.5 EU, were evaluated clinicopathologically.Results: Among 31 patients with PE 22 had underlying systemic diseases such as collagen vascular diseases, while nine had unknown aetiology. Evidence of glomerulonephritis was demonstrated in 14 patients. The clinical features were a history of dry cough and/or fine crackles in all 31 patients. Chest CT scans showed honeycombing in the lung bases in 26 patients. The histopathological features of the diseased lung tissues in all 11 autopsied cases were compatible with the usual interstitial pneumonia (UIP) pattern. Vasculitis was confirmed in bronchial arteries and/or pulmonary arterioles in five patients. The mortality was as high as 13 of the 31 patients. The causes of death were: deterioration of PF in five (two of whom were associated with pulmonary haemorrhage), lung cancer in two, pneumonia in four, and digestive tract bleeding in two. The survival rates in PF with MPO-ANCA-negative collagen vascular diseases, cryptogenic fibrosing alveolitis (CIA), and PF with positive MPO-ANCA, were compared. The 5-year survival rate in PF with positive MPO-ANCA was worse than in PF with MPO-ANCA-negative collagen vascular diseases and was the same for CIA.Conclusion: Although there was no correlation between MPO-ANCA titres and the activity of PE this study demonstrated that the presence of positive MPO-ANCA was an unfavorable prognostic factor in patients with PE.