Abstract
The purpose of this study was to evaluate the implications of the 2018 updated guideline for the diagnosis of idiopathic pulmonary fibrosis (IPF) in clinical practice compared to 2011 guideline. This study involved 535 patients including 339 IPF and 196 non-IPF, and we retrospectively evaluated CT classifications of usual interstitial pneumonia (UIP) by two guidelines. Interobserver agreement of 2018 criteria showed moderate reliability (κ = 0.53) comparable to 2011 (κ = 0.56) but interobserver agreement for probable UIP was fair (κ = 0.40). CT pattern of indeterminate for UIP was associated with better prognosis compared with the other groups (adjusted hazard ratio [HR] = 0.36, p < 0.001). Compared to possible UIP, probable UIP demonstrated a lower positive predictive value (PPV, 62.9% vs 65.8%). In analysis of patients with CT patterns of non-definite UIP, diagnosing IPF when CT pattern showed probable UIP with lymphocyte count ≤ 15% in BAL fluid, and either male sex or age ≥ 60 years showed a high specificity of 90.6% and a PPV of 80.8% in the validation cohort. The 2018 criteria provide better prognostic stratification than the 2011 in patients with possible UIP. BAL fluid analysis can improve the diagnostic certainty for IPF diagnosis in patients with probable UIP CT pattern.
Highlights
The purpose of this study was to evaluate the implications of the 2018 updated guideline for the diagnosis of idiopathic pulmonary fibrosis (IPF) in clinical practice compared to 2011 guideline
The present study showed that application of the latest 2018 diagnostic criteria for IPF proposed by ARS/European Respiratory Society (ERS)/ Japanese Respiratory Society (JRS)/ALAT resulted in the reclassification of patients categorized as having possible usual interstitial pneumonia (UIP) based on 2011 criteria into two categories, probable UIP and indeterminate for UIP
Diagnosing IPF based on a CT pattern of probable UIP on 2018 criteria showed increase in sensitivity, its specificity and PPV remained insufficient
Summary
The purpose of this study was to evaluate the implications of the 2018 updated guideline for the diagnosis of idiopathic pulmonary fibrosis (IPF) in clinical practice compared to 2011 guideline. Performing bronchoalveolar lavage (BAL) is conditionally recommended in group of patients with CT pattern of probable UIP, indeterminate for UIP and alternative diagnosis prior to surgical lung biopsy to distinguish IPF from the other alternative diagnosis including eosinophilic pneumonia, sarcoidosis, and hypersensitivity pneumonitis (HP). These recommendations, are conditional, and it is currently unclear how changes in the guidelines for IPF will affect their diagnostic performance in real clinical practice. The added diagnostic value of cellular analysis of BAL fluid was assessed in patients with CT patterns of probable UIP, indeterminate for UIP and alternative diagnoses following the guidline
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