MRC Human Immunology Unit, University of Oxford, Headington, Oxford, UK.
Interstitial Lung Disease Service, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.
BMJ Open Respir Res. 2021 Nov;8(1). doi: 10.1136/bmjresp-2021-000899.
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with poor prognosis. Identifying patients early may allow intervention which could limit progression. The 'indeterminate for usual interstitial pneumonia' (iUIP) CT pattern, defined in the 2018 IPF guidelines, could be a precursor to IPF but there is limited data on how patients with iUIP progress over time.
To evaluate the radiological progression of iUIP and explore factors linked to progression to IPF.
We performed a retrospective analysis of a lung fibrosis clinic cohort (n=230) seen between 2013 and 2017. Cases with iUIP were identified; first ever CTs for each patient found and categorised as 'non-progressor' or 'progressors' (the latter defined as increase in extent of disease or to 'definite' or 'probable' UIP CT pattern) during their follow-up. Lung function trends, haematological data and patient demographics were examined to explore disease evolution and potential contribution to progression.
48 cases with iUIP CT pattern were identified. Of these, 32 had follow-up CT scans, of which 23 demonstrated progression. 17 patients in this cohort were diagnosed with IPF over a mean (SD) period of 3.9 (±1.9) years. Monocyte (HR: 23, 95% CI: 1.6 to 340, p=0.03) and neutrophil levels (HR: 1.8, 95% CI: 1.3 to 2.3, p<0.001), obtained around the time of initial CT, were associated with progression to IPF using Cox proportional hazard modelling.
53% of our evaluable patients with iUIP progressed to IPF over a mean of 4 years. Monocyte and neutrophil levels at initial CT were significantly associated with progression in disease. These data provide a single-centre analysis of the evolution of patients with iUIP CT pattern, and first signal for potential factors associated with progression to IPF.
特发性肺纤维化（IPF）是一种进行性肺纤维化疾病，预后不良。早期识别患者可能有助于进行干预，从而限制疾病进展。2018 年 IPF 指南中定义的“不确定型寻常型间质性肺炎（iUIP）”CT 模式可能是 IPF 的前兆，但关于 iUIP 患者随时间推移如何进展的数据有限。
评估 iUIP 的放射学进展，并探讨与进展为 IPF 相关的因素。
我们对 2013 年至 2017 年间就诊的肺纤维化诊所队列（n=230）进行了回顾性分析。确定了 iUIP 病例；对每位患者的首次 CT 进行分类，分为“非进展者”或“进展者”（后者定义为疾病程度增加或 CT 模式为“明确”或“可能”UIP 模式）在他们的随访期间。检查肺功能趋势、血液学数据和患者人口统计学数据，以探讨疾病演变和潜在进展的贡献。
确定了 48 例 iUIP CT 模式病例。其中 32 例有随访 CT 扫描，其中 23 例显示进展。该队列中有 17 例患者在平均（SD）3.9（±1.9）年内被诊断为 IPF。在 Cox 比例风险模型中，初始 CT 时获得的单核细胞（HR：23，95%CI：1.6 至 340，p=0.03）和中性粒细胞水平（HR：1.8，95%CI：1.3 至 2.3，p<0.001）与进展为 IPF 相关。
我们可评估的 iUIP 患者中有 53%在平均 4 年内进展为 IPF。初始 CT 时的单核细胞和中性粒细胞水平与疾病进展显著相关。这些数据提供了 iUIP CT 模式患者演变的单中心分析，首次为与进展为 IPF 相关的潜在因素提供了信号。
