Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan.
Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Showa-ku, Nagoya, Aichi, Japan.
PLoS One. 2018 Mar 28;13(3):e0193608. doi: 10.1371/journal.pone.0193608. eCollection 2018.
Few studies have reported the diagnostic variability in patients with a possible usual interstitial pneumonia (UIP) pattern on high-resolution CT (HRCT) who underwent surgical lung biopsy (SLB), and the prognostic factors for these patients have not been fully evaluated. We retrospectively investigated the frequency of idiopathic pulmonary fibrosis (IPF) and prognostic factors in patients with possible UIP pattern on HRCT.
Consecutive patients who had a possible UIP pattern on HRCT, underwent SLB, and had a diagnosis of IIPs before SLB were retrospectively recruited from 10 hospitals. Diagnoses were made based on multidisciplinary discussion using the criteria for current IPF guidelines and multidisciplinary classification for IIPs in each hospital.
179 patients who underwent SLB were enrolled. The diagnoses were IPF in 91 patients (51%), unclassifiable IIPs in 47 (26%), idiopathic NSIP in 18 (10%), and chronic hypersensitivity pneumonia in 17 (9%). One-year FVC changes showed significant differences between IPF and non-IPF (-138.6 mL versus 18.2 mL, p = 0.014). Patients with IPF had a worse mortality than those with non-IPF (Logrank test, p = 0.025). Multivariable Cox regression analysis demonstrated that diagnoses of IPF (HR, 2.961; 95% CI, 1.183-7.410; p = 0.02), high modified MRC score (HR, 1.587; 95% CI, 1.003-2.510; p = 0.049), and low %FVC (HR, 0.972; 95% CI, 0.953-0.992; p = 0.005).
About a half of patients with a possible UIP pattern on HRCT had diagnoses other than IPF, and patients with IPF had a worse mortality than those with an alternative diagnosis. We reaffirmed that multidisciplinary discussion is crucial in patients with possible UIP pattern on HRCT.
很少有研究报告在接受高分辨率 CT（HRCT）手术肺活检（SLB）的可能特发性间质性肺炎（UIP）模式患者中存在诊断差异，并且这些患者的预后因素尚未得到充分评估。我们回顾性研究了 HRCT 上可能存在 UIP 模式的患者中特发性肺纤维化（IPF）的频率和预后因素。
从 10 家医院中回顾性招募了 HRCT 上可能存在 UIP 模式、接受 SLB 且在 SLB 前被诊断为间质性肺疾病（ILD）的连续患者。根据当前 IPF 指南的多学科讨论和每家医院的ILD 多学科分类标准，对诊断进行了判断。
共纳入 179 例接受 SLB 的患者。诊断为 IPF 的患者 91 例（51%），未分类的 ILD 47 例（26%），特发性非特异性间质性肺炎（NSIP）18 例（10%），慢性过敏性肺炎 17 例（9%）。IPF 组和非 IPF 组患者的 1 年 FVC 变化差异有统计学意义（-138.6mL 与 18.2mL，p=0.014）。IPF 患者的死亡率明显高于非 IPF 患者（Logrank 检验，p=0.025）。多变量 Cox 回归分析显示，IPF 诊断（HR，2.961；95%CI，1.183-7.410；p=0.02）、改良 MRC 评分较高（HR，1.587；95%CI，1.003-2.510；p=0.049）和低%FVC（HR，0.972；95%CI，0.953-0.992；p=0.005）是死亡的独立预后因素。
约一半的 HRCT 上可能存在 UIP 模式的患者存在非 IPF 诊断，且 IPF 患者的死亡率高于其他诊断患者。我们再次证实，在 HRCT 上可能存在 UIP 模式的患者中，多学科讨论至关重要。
