ANCA-associated lung fibrosis: Analysis of 17 patients
ANCA-associated lung fibrosis: Analysis of 17 patients
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DOI：
10.1016/j.rmed.2008.04.023
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发表时间：
2008-10-01
影响因子：
4.3
通讯作者：
Crestani, Bruno
中科院分区：
文献类型：
--
作者：
Foulon, Guillaume;Delaval, Philippe;Crestani, Bruno
在这项回顾性研究中，我们分析了17名表现为肺纤维化且ANCA检测呈阳性的患者。这组患者与12名ANCA试验阴性的IPF患者的对照组进行了比较。男15例，女2例，平均年龄66岁。8例为既往吸烟者，3例为现吸烟者，6例为非吸烟者。诊断时肺功能检查：总肺活量73%+/-18，肺活量82%+/-23，S用力呼气量88%+/-24，肺一氧化碳弥散量49%+/-2(%预测%)。支气管肺泡灌洗结果显示，随着中性粒细胞计数的增加，细胞密度增加。胸部高分辨率计算机断层扫描显示所有患者均有明显的纤维化，并伴有一定程度的毛玻璃密度。这些特征与对照组相似。显微镜下多血管炎(MPA)是ANCA阳性患者的主要并发症，发生在7例患者中(5例为抗髓过氧化物酶特异性)。6例肺纤维化先于MPa发生，1例同时诊断为MPa。在随访期间，10/17例患者死亡。死亡3例与脉管炎直接相关。我们的结论是，肺纤维化患者应该评估ANCA的存在。ANCA检测阳性的患者，特别是抗髓过氧化物酶阳性的患者，应该仔细监测，以检测显微镜下多血管炎的发生。(C)2008爱思唯尔有限公司。保留所有权利。
In this retrospective study, we analyzed 17 patients presenting with pulmonary fibrosis and a positive ANCA testing. This group was compared with a control group of 12 patients with IPF and negative ANCA testing. Patients were 15 males and 2 females, with a mean age of 66 years. Eight patients were past smokers, 3 current smokers and 6 non-smokers. Lung function tests at diagnosis were as follows (% predicted): total lung capacity 73% +/- 18, vital capacity 82% +/- 23, forced expiratory volume in 1 s (FEV(1)) 88% +/- 24, carbon monoxide diffusion capacity of the lung 49% +/- 2 (% predicted). Bronchoalveolar lavage results showed an increased cellularity with increased neutrophils counts. High resolution computed tomography of the chest showed prominent fibrosis with some degree of ground-glass attenuation in all patients. These characteristics were similar to the control group. Microscopic polyangiitis (MPA) was a major complicating event in ANCA-positive patients, occurring in 7 patients (anti-myeloperoxidase specificity in 5 patients). Pulmonary fibrosis predated occurrence of MPA in 6 patients and was diagnosed concomitantly with MPA in 1 patient. During the follow-up, 10/17 patients died. The death was directly related to vasculitis in 3 patients. We conclude that patients with pulmonary fibrosis should be evaluated for the presence of ANCA. Patients with positive ANCA testing, particularly if anti-myeloperoxidase, should be carefully monitored to detect the occurrence of microscopic polyangiitis. (C) 2008 Elsevier Ltd. All rights reserved.