Marinescu Daniel-Costin, Hague Cameron J, Muller Nestor L, Murphy Darra, Churg Andrew, Wright Joanne L, Al-Arnawoot Amna, Bilawich Ana-Maria, Bourgouin Patrick, Cox Gerard, Durand Celine, Elliot Tracy, Ellis Jennifer, Fisher Jolene H, Fladeland Derek, Grant-Orser Amanda, Goobie Gillian C, Guenther Zachary, Haider Ehsan, Hambly Nathan, Huynh James, Johannson Kerri A, Karjala Geoffrey, Khalil Nasreen, Kolb Martin, Leipsic Jonathon, Lok Stacey, MacIsaac Sarah, McInnis Micheal, Manganas Helene, Marcoux Veronica, Mayo John, Morisset Julie, Scallan Ciaran, Sedlic Tony, Shapera Shane, Sun Kelly, Tan Victoria, Wong Alyson W, Zheng Boyang, Ryerson Christopher J
Department of Medicine, University of British Columbia, Vancouver, BC, Canada; Centre for Heart Lung Innovation, St. Paul's Hospital, Vancouver, BC, Canada.
Department of Radiology, University of British Columbia, Vancouver, BC, Canada.
Chest. 2023 Dec;164(6):1466-1475. doi: 10.1016/j.chest.2023.07.068. Epub 2023 Aug 2.
Clinical practice guidelines separately describe radiologic patterns of usual interstitial pneumonia (UIP) and fibrotic hypersensitivity pneumonitis (fHP), without direction on whether or how to apply these approaches concurrently within a single patient.
How can we integrate guideline-defined radiologic patterns to diagnose interstitial lung disease (ILD) and what are the pitfalls associated with described patterns that require reassessment in future guidelines?
Patients from the Canadian Registry for Pulmonary Fibrosis underwent detailed reevaluation in standardized multidisciplinary discussion. CT scan features were quantified by chest radiologists masked to clinical data, and guideline-defined patterns were assigned. Clinical data then were provided to the radiologist and an ILD clinician, who jointly determined the leading diagnosis.
Clinical-radiologic diagnosis in 1,593 patients was idiopathic pulmonary fibrosis (IPF) in 26%, fHP in 12%, connective tissue disease-associated ILD (CTD-ILD) in 34%, idiopathic pneumonia with autoimmune features in 12%, and unclassifiable ILD in 10%. Typical and probable UIP patterns corresponded to a diagnosis of IPF in 66% and 57% of patients, respectively. Typical fHP pattern corresponded to an fHP clinical diagnosis in 65% of patients, whereas compatible fHP was nonspecific and associated with CTD-ILD or IPAF in 48% of patients. No pattern ruled out CTD-ILD. Gas trapping affecting > 5% of lung parenchyma on expiratory imaging was an important feature broadly separating compatible and typical fHP from other patterns (sensitivity, 0.77; specificity, 0.91).
An integrated approach to guideline-defined UIP and fHP patterns is feasible and supports > 5% gas trapping as an important branch point. Typical or probable UIP and typical fHP patterns have moderate predictive values for a corresponding diagnosis of IPF and fHP, although occasionally confounded by CTD-ILD; compatible fHP is nonspecific.
临床实践指南分别描述了普通型间质性肺炎（UIP）和纤维化性过敏性肺炎（fHP）的放射学模式，但对于是否以及如何在同一患者中同时应用这些方法未给出指导。
我们如何整合指南定义的放射学模式来诊断间质性肺疾病（ILD），以及在未来指南中需要重新评估的所述模式相关的陷阱有哪些？
来自加拿大肺纤维化登记处的患者在标准化多学科讨论中接受了详细的重新评估。胸部放射科医生在不知道临床数据的情况下对CT扫描特征进行量化，并指定指南定义的模式。然后将临床数据提供给放射科医生和ILD临床医生，他们共同确定主要诊断。
1593例患者的临床放射学诊断为特发性肺纤维化（IPF）占26%，fHP占12%，结缔组织病相关ILD（CTD-ILD）占34%，具有自身免疫特征的特发性肺炎占12%，无法分类的ILD占10%。典型和可能的UIP模式分别在66%和57%的患者中对应IPF诊断。典型fHP模式在65%的患者中对应fHP临床诊断，而符合fHP是非特异性的，在48%的患者中与CTD-ILD或IPAF相关。没有模式可以排除CTD-ILD。呼气成像时影响>5%肺实质的气体潴留是一个重要特征，可将符合和典型fHP与其他模式广泛区分开来（敏感性为0.77；特异性为0.91）。
对指南定义的UIP和fHP模式采用综合方法是可行的，并支持>5%的气体潴留作为一个重要的分支点。典型或可能的UIP和典型fHP模式对IPF和fHP的相应诊断具有中等预测价值，尽管偶尔会被CTD-ILD混淆；符合fHP是非特异性的。
