Department of Pulmonary Medicine, Inselspital, Bern University Hospital, University of Bern, 3010, Bern, Switzerland.
Schlosspraxis Schwarzenburg, Schwarzenburg, Switzerland.
Respir Res. 2021 Apr 23;22(1):120. doi: 10.1186/s12931-021-01727-7.
The differential diagnosis fibrotic hypersensitivity pneumonitis (HP) versus idiopathic pulmonary fibrosis (IPF) is important but challenging. Recent diagnostic guidelines for HP emphasize including multidisciplinary discussion (MDD) in the diagnostic process, however MDD is not comprehensively available. We aimed to establish the diagnostic accuracy and prognostic validity of a previously proposed HP diagnostic algorithm that foregoes MDD.
We tested the algorithm in patients with an MDD diagnosis of fibrotic HP or IPF (case control study) and determined diagnostic test performances for diagnostic confidences of ≥ 90% and ≥ 70%. Prognostic validity was established using Cox proportional hazards models.
Thirty-one patients with fibrotic HP and 50 IPF patients were included. The algorithm-derived ≥ 90% confidence level for HP had high specificity (0.94, 95% confidence interval [CI] 0.83-0.99), but low sensitivity (0.35 [95%CI 0.19-0.55], J-index 0.29). Test performance was improved for the ≥ 70% confidence level (J-index 0.64) with a specificity of 0.90 (95%CI 0.78-0.97), and a sensitivity of 0.74 (95%CI 0.55-0.88). MDD fibrotic HP diagnosis was strongly associated with lower risk of death (adjusted hazard ratio [HR] 0.10 [0.01-0.92], p = 0.04), whereas the algorithm-derived ≥ 70% and ≥ 90% confidence diagnoses were not significantly associated with survival (adjusted HR 0.37 [0.07-1.80], p = 0.22, and adjusted HR 0.41 [0.05-3.25], p = 0.39, respectively).
The algorithm-derived ≥ 70% diagnostic confidence had satisfactory test performance for MDD-HP diagnosis, with insufficient sensitivity for ≥ 90% confidence. The lowest risk of death in the MDD-derived HP diagnosis validates the reference standard and suggests that a diagnostic algorithm not including MDD, might not replace the latter.
纤维化性过敏性肺炎（HP）与特发性肺纤维化（IPF）的鉴别诊断很重要，但具有挑战性。最近 HP 的诊断指南强调在诊断过程中纳入多学科讨论（MDD），但 MDD 并不能全面提供。我们旨在建立一个先前提出的放弃 MDD 的 HP 诊断算法的诊断准确性和预后有效性。
我们在接受 MDD 纤维化性 HP 或 IPF 诊断的患者中测试了该算法（病例对照研究），并确定了诊断置信度为≥90%和≥70%的诊断测试性能。使用 Cox 比例风险模型确定预后有效性。
纳入 31 例纤维化性 HP 患者和 50 例 IPF 患者。该算法得出的 HP 置信度≥90%具有很高的特异性（0.94，95%置信区间[CI]0.83-0.99），但敏感性较低（0.35[95%CI 0.19-0.55]，J 指数 0.29）。对于置信度≥70%的水平，测试性能得到改善（J 指数 0.64），特异性为 0.90（95%CI 0.78-0.97），敏感性为 0.74（95%CI 0.55-0.88）。MDD 纤维化性 HP 诊断与较低的死亡风险密切相关（调整后的危险比[HR]0.10[0.01-0.92]，p=0.04），而算法得出的≥70%和≥90%置信诊断与生存无关（调整后的 HR 0.37[0.07-1.80]，p=0.22，调整后的 HR 0.41[0.05-3.25]，p=0.39）。
该算法得出的≥70%诊断置信度对 MDD-HP 诊断具有令人满意的测试性能，但对于≥90%置信度的敏感性不足。在 MDD 诊断的 HP 中，死亡风险最低验证了参考标准，并表明不包括 MDD 的诊断算法可能无法替代后者。
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