Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan.
Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan.
PLoS One. 2018 Jun 21;13(6):e0199659. doi: 10.1371/journal.pone.0199659. eCollection 2018.
Although a possible association among myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA), microscopic polyangiitis (MPA), and idiopathic pulmonary fibrosis (IPF) has been suggested, the clinical significance of MPO-ANCA in idiopathic interstitial pneumonias (IIPs), including IPF and non-IPF, remains unclear. We aimed to investigate the frequency of MPO-ANCA positivity, as well as MPA incidence and risk factors for development in patients initially diagnosed with IIP.
We retrospectively analysed 305 consecutive patients who were initially diagnosed as IIP and had MPO-ANCA results available.
Of the 305 patients, 26 (8.5%) were MPO-ANCA-positive. Baseline characteristics were similar between the MPO-ANCA-positive and -negative patients. The cumulative 5-year MPA incidence was 24.3% in the MPO-ANCA-positive patients and 0% in the -negative patients (P < 0.0001). MPO-ANCA was positive in 15 of 133 (11.3%) patients initially diagnosed with IPF and in 11 of 172 (6.3%) patients initially diagnosed with non-IPF (P = 0.56), with cumulative 5-year MPA incidence of 6.2% and 1.0%, respectively (P = 0.10). Multivariate analysis revealed that UIP pattern on HRCT (HR = 3.20, P < 0.01) and no treatment for IIP (HR = 3.52, P < 0.01) were independently associated with MPA development in MPO-ANCA-positive patients.
MPO-ANCA positivity was uncommon, but was associated with subsequent MPA development in patients initially diagnosed with IIP, including both IPF and non-IPF cases. The study suggested that attention should be paid to MPA development in MPO-ANCA-positive IIP patients with UIP pattern on HRCT and those without treatment for IIP.
虽然已经提出髓过氧化物酶-抗中性粒细胞胞质抗体（MPO-ANCA）、显微镜下多血管炎（MPA）和特发性肺纤维化（IPF）之间可能存在关联，但 MPO-ANCA 在特发性间质性肺炎（IIP）中的临床意义，包括 IPF 和非 IPF，仍不清楚。我们旨在研究最初诊断为 IIP 的患者中 MPO-ANCA 阳性的频率，以及 MPA 的发病率和发展的危险因素。
我们回顾性分析了 305 例连续的最初诊断为 IIP 且 MPO-ANCA 结果可用的患者。
在 305 例患者中，有 26 例（8.5%）为 MPO-ANCA 阳性。MPO-ANCA 阳性和阴性患者的基线特征相似。MPO-ANCA 阳性患者的 5 年累积 MPA 发病率为 24.3%，而 MPO-ANCA 阴性患者为 0%（P < 0.0001）。最初诊断为 IPF 的 133 例患者中有 15 例（11.3%）和最初诊断为非 IPF 的 172 例患者中有 11 例（6.3%）为 MPO-ANCA 阳性（P = 0.56），5 年累积 MPA 发病率分别为 6.2%和 1.0%（P = 0.10）。多变量分析显示，HRCT 上 UIP 模式（HR = 3.20，P < 0.01）和 IIP 无治疗（HR = 3.52，P < 0.01）与 MPO-ANCA 阳性患者的 MPA 发展独立相关。
MPO-ANCA 阳性并不常见，但与最初诊断为 IIP 的患者，包括 IPF 和非 IPF 患者的后续 MPA 发展相关。该研究表明，应注意 HRCT 上 UIP 模式和无 IIP 治疗的 MPO-ANCA 阳性 IIP 患者的 MPA 发展。
