Department of Internal Medicine (IV), Osaka Medical College, Daigaku-Machi 2-7, Takatsuki, Osaka, 569-8686, Japan.
Department of Medical Statistics, Research and Development Center, Osaka Medical College, Takatsuki, Osaka, Japan.
Sci Rep. 2021 Jan 15;11(1):1490. doi: 10.1038/s41598-021-81311-7.
The prognosis of microscopic polyangiitis (MPA) with interstitial lung disease (ILD) is significantly worse than that of MPA without ILD. However, the clinical characteristics in MPA-ILD, especially poor prognostic factors, are not elucidated. We evaluated demographic, clinical, laboratory, and radiological findings, treatments, and outcomes of 80 patients with MPA, and investigated prognostic factors of respiratory-related death in patients with myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) positive MPA-ILD. Ground-glass opacity and fibrosis were evaluated as scores on high-resolution computed tomography (HRCT). The presence of ILD was consistent with a high risk of respiratory-related death (hazard ratio, 4.8; P = 0.04). Multivariable logistic regression analyses using propensity scoring showed right or left lower lobe fibrosis score to be significantly associated with respiratory-related death (P = 0.0005 and 0.0045, respectively). A right or left lower lobe fibrosis score ≥ 2, indicating the presence of honeycombing at 1 cm above the diaphragm, was determined to be the best cut-off value indicating a poor prognosis. The 5-year survival rate was significantly lower in patients with right or left lower lobe fibrosis score ≥ 2 (survival rates: 37% and 19%, respectively) than those with a score < 2 (71% and 68%, respectively) (P = 0.002 and 0.0007, respectively). These findings suggest that the presence of honeycomb lesions in bilateral lower lobes on chest HRCT was associated with respiratory-related death in patients with MPO-ANCA positive MPA-ILD.
显微镜下多血管炎（MPA）合并间质性肺病（ILD）的预后明显差于无ILD 的 MPA。然而，MPA-ILD 的临床特征，尤其是不良预后因素，尚未阐明。我们评估了 80 例 MPA 患者的人口统计学、临床、实验室和影像学表现、治疗方法和结局，并研究了髓过氧化物酶（MPO）-抗中性粒细胞胞质抗体（ANCA）阳性 MPA-ILD 患者呼吸相关死亡的预后因素。高分辨率计算机断层扫描（HRCT）评估磨玻璃影和纤维化的评分。ILD 的存在与呼吸相关死亡的高风险一致（风险比，4.8；P = 0.04）。使用倾向评分的多变量逻辑回归分析显示，右或左肺下叶纤维化评分与呼吸相关死亡显著相关（P = 0.0005 和 0.0045）。右或左肺下叶纤维化评分≥2 表明膈肌上 1cm 处存在蜂窝肺，被确定为预后不良的最佳截断值。右或左肺下叶纤维化评分≥2 的患者 5 年生存率明显低于评分<2 的患者（生存率：分别为 37%和 19%；分别为 71%和 68%）（P = 0.002 和 0.0007）。这些发现表明，胸部 HRCT 双侧下肺的蜂窝状病变与 MPO-ANCA 阳性 MPA-ILD 患者的呼吸相关死亡相关。
