Division of Pulmonary and Critical Care Medicine, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75219, USA.
Department of Radiology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75219, USA.
BMC Pulm Med. 2023 Jun 22;23(1):224. doi: 10.1186/s12890-023-02523-3.
The distinction between hypersensitivity pneumonitis (HP) and idiopathic pulmonary fibrosis (IPF) was thought to be important due to the difference in mortality between the conditions as well as the response to treatment. However, recent work suggests that the clinical diagnosis may matter less than certain radiographic features, namely usual interstitial pneumonia (UIP) pattern. The purpose of this study is to evaluate whether radiographic honeycombing is more predictive of transplant-free survival (TFS) than other clinical, radiographic, or histologic findings that distinguish HP from IPF in the current guidelines and to evaluate the impact of radiographic honeycombing on the efficacy of immunosuppression in fibrotic HP.
We retrospectively identified IPF and fibrotic HP patients evaluated between 2003 and 2019. Univariable and multivariable logistic regression was performed for patients with fibrotic HP and IPF to evaluate TFS. To assess the impact of treatment with immunosuppression on TFS in fibrotic HP, a cox proportional hazard model adjusted for known predictors of survival in HP including age, gender, and baseline pulmonary function testing results was constructed, and p-interaction for the presence of honeycombing on high resolution computed tomography and use of immunosuppression was calculated.
Our cohort included 178 with IPF and 198 with fibrotic HP. In a multivariable analysis, the presence of honeycombing had a greater impact on the TFS than the diagnosis of HP vs. IPF. Among the criteria used in the HP diagnostic guidelines, only typical HP scan impacted survival in a multivariable model, while identification of antigen and surgical lung biopsy findings had no impact on survival. We identified a trend toward worse survival on immunosuppression in those with HP with radiographic honeycombing.
Our data suggests that honeycombing and baseline pulmonary function testing have a greater impact on TFS than the clinical diagnosis of IPF vs. fibrotic HP and that radiographic honeycombing is a predictor of poor TFS in fibrotic HP. We suggest that invasive diagnostic testing including surgical lung biopsy may not be useful in predicting mortality in HP patients with honeycombing and may potentially increase risk of immunosuppression.
由于这两种疾病的死亡率以及对治疗的反应不同，人们曾认为区分过敏性肺炎（HP）和特发性肺纤维化（IPF）很重要。然而，最近的研究表明，临床诊断可能不如某些影像学特征重要，即常见间质性肺炎（UIP）模式。本研究旨在评估在当前指南中，与 HP 区分开来的 IPF 相比，影像学蜂窝状改变是否更能预测无移植生存（TFS），并评估影像学蜂窝状改变对纤维化 HP 中免疫抑制疗效的影响。
我们回顾性地确定了 2003 年至 2019 年期间评估的 IPF 和纤维化 HP 患者。对纤维化 HP 和 IPF 患者进行单变量和多变量逻辑回归，以评估 TFS。为了评估纤维化 HP 中免疫抑制治疗对 TFS 的影响，构建了一个 Cox 比例风险模型，该模型调整了 HP 中生存的已知预测因素，包括年龄、性别和基线肺功能测试结果，并计算了高分辨率 CT 上存在蜂窝状改变和使用免疫抑制的交互作用 p 值。
我们的队列包括 178 例 IPF 和 198 例纤维化 HP。在多变量分析中，与 HP 与 IPF 的诊断相比，蜂窝状的存在对 TFS 的影响更大。在 HP 诊断指南中使用的标准中，只有典型的 HP 扫描在多变量模型中影响生存，而识别抗原和手术肺活检结果对生存没有影响。我们发现，在存在影像学蜂窝状改变的 HP 患者中，免疫抑制的生存预后较差。
我们的数据表明，蜂窝状改变和基线肺功能测试对 TFS 的影响大于 IPF 与纤维化 HP 的临床诊断，影像学蜂窝状改变是纤维化 HP 患者 TFS 不良的预测指标。我们建议，对于有蜂窝状改变的 HP 患者，包括手术肺活检在内的侵入性诊断测试可能无法预测死亡率，并且可能会增加免疫抑制的风险。
