Performance of a diagnostic algorithm for fibrotic hypersensitivity pneumonitis. A case-control study.

Guler, Sabina A; Wohlfarth, Eva; Berezowska, Sabina; Geiser, Thomas K; Ebner, Lukas; Funke-Chambour, Manuela (2021). Performance of a diagnostic algorithm for fibrotic hypersensitivity pneumonitis. A case-control study. Respiratory research, 22(1), p. 120. BioMed Central 10.1186/s12931-021-01727-7

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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.

Item Type:

Journal Article (Original Article)


04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Pneumology
04 Faculty of Medicine > Department of Radiology, Neuroradiology and Nuclear Medicine (DRNN) > Institute of Diagnostic, Interventional and Paediatric Radiology
04 Faculty of Medicine > Service Sector > Institute of Pathology

UniBE Contributor:

Guler, Sabina Anna, Wohlfarth, Eva, Berezowska, Sabina Anna, Geiser, Thomas (A), Ebner, Lukas, Funke-Chambour, Manuela


600 Technology > 610 Medicine & health
500 Science > 570 Life sciences; biology




BioMed Central




Maria de Fatima Henriques Bernardo

Date Deposited:

07 May 2021 12:18

Last Modified:

29 Mar 2023 23:37

Publisher DOI:


PubMed ID:


Uncontrolled Keywords:

Algorithms Alveolitis Diagnosis Environmental Exposure Extrinsic Allergic Pulmonary Fibrosis




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