Diagnostic Likelihood Thresholds That Define a Working Diagnosis of Idiopathic Pulmonary Fibrosis

Walsh, Simon L.F.; Lederer, David J.; Ryerson, Christopher J.; Kolb, Martin; Maher, Toby M.; Nusser, Richard; Poletti, Venerino; Richeldi, Luca; Vancheri, Carlo; Wilsher, Margaret; Αντωνίου, Κατερίνα; Behr, Jüergen; Bendstrup, Elisabeth; Brown, Kevin K.; Corte, Tamera J.; Cottin, Vincent; Crestani, Bruno; Flaherty, Kevin R.; Glaspole, Ian; Grutters, Jan C.; Inoue, Yoshikazu; Kondoh, Yasuhiro; Kreuter, Michael; Johannson, Kerri A.; Ley, Brett; Martínez, Fernando J.; Molina-Molina, María; Morais, António; Nunès, Hilario; Raghu, Ganesh; Selman, Moisés; Spagnolo, Paolo; Taniguchi, Hiroyuki; Tomassetti, Sara; Valeyre, Dominique; Wijsenbeek, Marlies; Wuyts, Wim; Wells, Athol U.

doi:10.1164/rccm.201903-0493oc

Cited by 67 publications

(41 citation statements)

References 17 publications

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“…We categorized diagnoses as definite (≥90% confidence), high-confidence (80–89%), moderate-confidence (70–79%), and low-confidence (51–69%) diagnoses. This approach is supported by recent studies suggesting the potential therapeutic and prognostic utility of assigning diagnostic confidence in this manner ( 160 , 161 ).…”

Section: Diagnostic Criteriamentioning

confidence: 68%

“…Patients with any other combination of exposure history, HRCT pattern, and BAL results should undergo an MDD that includes an experienced expert in ILD (pulmonologist), a chest radiologist, and, if transbronchial lung biopsies were performed at the time of BAL, a pathologist familiar with histopathological features of interstitial pneumonias and HP. Additional histopathological sampling should be considered after the MDD in some patients with a high-confidence diagnosis, moderate-confidence diagnosis, or low-confidence diagnosis or in patients for whom an alternative diagnosis has not been established ( 161 ). A low-confidence diagnosis may be adequate in patients for whom the differential diagnosis has been sufficiently narrowed such that further investigations are unlikely to alter management, when invasive testing has unacceptable risks, or when such tests are declined by the patient.…”

Section: Diagnostic Criteriamentioning

confidence: 99%

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Diagnosis of Hypersensitivity Pneumonitis in Adults: An Official ATS/JRS/ALAT Clinical Practice Guideline

Raghu¹,

Rémy‐Jardin²,

Ryerson³

et al. 2020

Am J Respir Crit Care Med

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679

684

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Background: This guideline addresses the diagnosis of hypersensitivity pneumonitis (HP). It represents a collaborative effort among the American Thoracic Society, Japanese Respiratory Society, and Asociación Latinoamericana del Tórax. Methods: Systematic reviews were performed for six questions. The evidence was discussed, and then recommendations were formulated by a multidisciplinary committee of experts in the field of interstitial lung disease and HP using the GRADE (Grading of Recommendations, Assessment, Development, and Evaluation) approach. Results: The guideline committee defined HP, and clinical, radiographic, and pathological features were described. HP was classified into nonfibrotic and fibrotic phenotypes. There was limited evidence that was directly applicable to all questions. The need for a thorough history and a validated questionnaire to identify potential exposures was agreed on. Serum IgG testing against potential antigens associated with HP was suggested to identify potential exposures. For patients with nonfibrotic HP, a recommendation was made in favor of obtaining bronchoalveolar lavage (BAL) fluid for lymphocyte cellular analysis, and suggestions for transbronchial lung biopsy and surgical lung biopsy were also made. For patients with fibrotic HP, suggestions were made in favor of obtaining BAL for lymphocyte cellular analysis, transbronchial lung cryobiopsy, and surgical lung biopsy. Diagnostic criteria were established, and a diagnostic algorithm was created by expert consensus. Knowledge gaps were identified as future research directions. Conclusions: The guideline committee developed a systematic approach to the diagnosis of HP. The approach should be reevaluated as new evidence accumulates.

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Section: Diagnostic Criteriamentioning

confidence: 68%

Section: Diagnostic Criteriamentioning

confidence: 99%

Diagnosis of Hypersensitivity Pneumonitis in Adults: An Official ATS/JRS/ALAT Clinical Practice Guideline

Raghu¹,

Rémy‐Jardin²,

Ryerson³

et al. 2020

Am J Respir Crit Care Med

Self Cite

679

684

View full text Add to dashboard Cite

show abstract

“…The base finding of our study was that a noticeable proportion of IPF patients in our overall cohort (16.5%) and more than half of the patients with a possible UIP on HRCT received IPF diagnosis and antifibrotic treatment without requiring SLB, suggesting a general tendency to diagnose IPF avoiding invasive procedures. These findings are in line with a recent case-cohort study by Walsh et al, 9 where SLB was requested by clinicians in only a minority of cases given a provisional high-confidence diagnosis of IPF (70-89% diagnostic likelihood) and antifibrotics prescription was deemed appropriate in 63% of cases with a provisional highconfidence diagnosis of IPF and no SLB requested. Interestingly, no patients from one of the three centres fell into this diagnostic category, presumably due to the multidisciplinary team requiring biopsy in all patients with radiological possible UIP.…”

Section: Discussionsupporting

confidence: 89%

Disease progression across the spectrum of idiopathic pulmonary fibrosis: A multicentre study

et al. 2020

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Background and objective: In clinical practice, a working diagnosis of IPF may be performed to provide effective antifibrotic treatment to patients who cannot undergo SLB. In this study, we compared the disease course across IPF diagnostic categories in a real-life clinical setting to clarify the appropriateness of a working diagnosis of IPF and treatment initiation in these patients. Methods: Longitudinal data from IPF patients receiving antifibrotic treatment (pirfenidone or nintedanib) were retrospectively collected at three tertiary centres in Italy. Univariate and multivariate analyses were performed to compare time to death and to a composite endpoint of disease progression between two diagnostic subgroups, that is, patients with UIP on HRCT and/or SLB, and patients with possible UIP and no histological confirmation. Results: A total of 249 IPF patients were included in the analysis. Among patients with a possible UIP pattern on HRCT, 41 (55%) were prescribed antifibrotic treatment (either nintedanib or pirfenidone) despite absence of histological confirmation. This group demonstrated similar mortality and disease progression as compared to patients with a definite diagnosis of IPF as per diagnostic guidelines (log-rank test P = 0.771 and P = 0.139, respectively). Such findings were confirmed on multivariate analysis (HR: 1.19, 95% CI: 0.49-2.89, P = 0.7 for death; HR: 1.42, 95% CI: 0.83-2.44, P = 0.201 for disease progression). Conclusion: In patients receiving antifibrotics following a working diagnosis of IPF, disease progression rates were similar to patients with a confident diagnosis of IPF according to consensus guidelines, supporting the rationale for treatment initiation in these patients by expert multidisciplinary teams.

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“…This is particularly true in the context of a probable UIP pattern on HRCT, in line with Fleischner Society recommendations [6]. A recent study involving 404 physicians from around the globe found that 63% were prepared to prescribe treatment for patients with a “working diagnosis of IPF” without requesting an SLB [11]. In the UK, it is estimated that only 13% of IPF diagnoses included an SLB [12].…”

Section: Introductionmentioning

confidence: 80%

Transbronchial Lung Cryobiopsy in Idiopathic Pulmonary Fibrosis: A State of the Art Review

et al. 2019

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Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease associated with significant morbidity and mortality. The diagnosis of IPF involves a combination of clinical history, radiological imaging and examination of histopathological samples in appropriate cases. Historically, transbronchial biopsy (TBB) has been used to obtain histological samples; however this lacks diagnostic accuracy. At present, surgical lung biopsy (SLB) is the gold standard technique for obtaining specimen samples; however this carries a significant mortality risk. Transbronchial lung cryobiopsy (TBLC) is a new technique that has been pioneered in the management of lung malignancy and offers a potential alternative to SLB. The technique employs a freezing probe, which is used to obtain lung tissue samples that are larger and better quality than traditional TBB samples. This affords TBLC an estimated diagnostic yield of 80% in interstitial lung disease. However, with limited evidence directly comparing TBLC to SLB, the diagnostic accuracy of the procedure has been uncertain. Common complications of TBLC include pneumothorax and bleeding. Mortality in TBLC is low compared with SLB, with exacerbation of IPF frequently reported as the cause. TBLC represents an exciting potential option in the diagnostic pathway in IPF; however its true value has yet to be determined.

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Diagnostic Likelihood Thresholds That Define a Working Diagnosis of Idiopathic Pulmonary Fibrosis

Cited by 67 publications

References 17 publications

Diagnosis of Hypersensitivity Pneumonitis in Adults: An Official ATS/JRS/ALAT Clinical Practice Guideline

Diagnosis of Hypersensitivity Pneumonitis in Adults: An Official ATS/JRS/ALAT Clinical Practice Guideline

Disease progression across the spectrum of idiopathic pulmonary fibrosis: A multicentre study

Transbronchial Lung Cryobiopsy in Idiopathic Pulmonary Fibrosis: A State of the Art Review

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