scholarly journals Practical application and validation of the 2018 ATS/ERS/JRS/ALAT and Fleischner Society guidelines for the diagnosis of idiopathic pulmonary fibrosis

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Angela R. Shih ◽  
Chayanin Nitiwarangkul ◽  
Brent P. Little ◽  
Benjamin W. Roop ◽  
Sreyankar Nandy ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interobserver Variability ◽  
High Specificity ◽  
Classification Performance ◽  
Accurate Diagnosis ◽  
Practical Application ◽  
Test Characteristics ◽  
High Positive Predictive Value ◽  
Usual Interstitial Pneumonitis

Abstract Background Accurate diagnosis of idiopathic pulmonary fibrosis (IPF) is essential to inform prognosis and treatment. In 2018, the ATS/ERS/JRS/ALAT and Fleischner Society released new diagnostic guidelines for usual interstitial pneumonitis (UIP)/IPF, adding Probable UIP as a CT category based on prior studies demonstrating this category had relatively high positive predictive value (PPV) for histopathologic UIP/Probable UIP. This study applies the 2018 ATS/ERS/JRS/ALAT and Fleischner Society guidelines to determine test characteristics of CT categories in academic clinical practice. Methods CT and histopathology were evaluated by three thoracic radiologists and two thoracic pathologists. Comparison of consensus categorization by the 2018 ATS and Fleischner Society guidelines by CT and histopathology was performed. Results Of patients with CT UIP, 87% (PPV, 95% CI: 60–98%) had histopathologic UIP with 97% (CI: 90–100%) specificity. Of patients with CT Probable UIP, 38% (PPV, CI: 14–68%) had histopathologic UIP and 46% (PPV, CI: 19–75%) had either histopathologic UIP or Probable UIP, with 88% (CI: 77–95%) specificity. Patients with CT Indeterminate and Alternative Diagnosis had histopathologic UIP in 27% (PPV, CI: 6–61%) and 21% (PPV, CI: 11–33%) of cases with specificities of 90% (CI: 80–96%) and 25% (CI: 16–37%). Interobserver variability (kappa) between radiologists ranged 0.32–0.81. Conclusions CT UIP and Probable UIP have high specificity for histopathologic UIP, and CT UIP has high PPV for histopathologic UIP. PPV of CT Probable UIP was 46% for combined histopathologic UIP/Probable UIP. Our results indicate that additional studies are needed to further assess and refine the guideline criteria to improve classification performance.

scholarly journals Diagnostic and prognostic implications of 2018 guideline for the diagnosis of idiopathic pulmonary fibrosis in clinical practice

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Jooae Choe ◽  
Byoung Soo Kwon ◽  
Kyung-Hyun Do ◽  
Hee Sang Hwang ◽  
Jin Woo Song ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interobserver Agreement ◽  
Usual Interstitial Pneumonia ◽  
High Specificity ◽  
Fluid Analysis ◽  
Diagnostic Certainty ◽  
Prognostic Stratification ◽  
Prognostic Implications

AbstractThe 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.

scholarly journals The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review

2015 ◽  
Vol 24 (135) ◽  
pp. 69-77 ◽  
Author(s):  
Sara Tomassetti ◽  
Sara Piciucchi ◽  
Paola Tantalocco ◽  
Alessandra Dubini ◽  
Venerino Poletti
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interstitial Pneumonia ◽  
Multidisciplinary Approach ◽  
Accurate Diagnosis ◽  
Diagnostic Assessment ◽  
Patient Case ◽  
Specialist Nurse ◽  
Unnecessary Testing ◽  
Worse Prognosis

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressively fibrosing interstitial pneumonia that is associated with a significantly worse prognosis than other forms of chronic interstitial pneumonia. An early and accurate diagnosis of IPF is important to enable the initiation of disease-specific therapies, which have the potential to reduce disease progression, and the avoidance of inappropriate and potentially harmful drugs. Establishing an accurate diagnosis of IPF can be challenging. Recent studies and international guidelines advocate the importance of a multidisciplinary team (MDT) in the initial diagnostic assessment of patients with suspected IPF. Typical MDT members include a pulmonologist, a radiologist and a pathologist, with further input from a thoracic surgeon, a rheumatologist, a specialist nurse and an occupational physician where appropriate. Multidisciplinary diagnosis is considered the gold standard because it can improve the accuracy of diagnosis of IPF, avoid unnecessary testing (e.g. lung biopsy), and optimise patient management. Here we highlight the strengths and limitations of the multidisciplinary approach to IPF diagnosis through MDT discussion of two patient cases.

scholarly journals Variable utility of mosaic attenuation to distinguish fibrotic hypersensitivity pneumonitis from idiopathic pulmonary fibrosis

2019 ◽  
Vol 54 (1) ◽  
pp. 1900531 ◽  
Author(s):  
Joseph Barnett ◽  
Philip L. Molyneaux ◽  
Bhavin Rawal ◽  
Rezaur Abdullah ◽  
Samanjit S. Hare ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Hypersensitivity Pneumonitis ◽  
High Specificity ◽  
Diagnostic Feature ◽  
Low Density ◽  
Air Trapping ◽  
International Guidelines ◽  
Frequent Finding ◽  
Important Diagnostic Feature

BackgroundMosaic attenuation on computed tomography (CT) has been identified in international guidelines as an important diagnostic feature of fibrotic hypersensitivity pneumonitis (FHP) as opposed to idiopathic pulmonary fibrosis (IPF). However, mosaic attenuation comprises several different radiological signs (low-density lobules, preserved lobules, air trapping and the so-called “headcheese sign”) which may have differing diagnostic utility. Furthermore, the extent of mosaic attenuation required to distinguish these two diagnoses is uncertain and thresholds of mosaic attenuation from international guidelines have not been validated.MethodsInspiratory and expiratory CT scans were evaluated by two readers in 102 patients (IPF n=57; FHP n=45) using a semiquantitative scoring system for mosaic attenuation. Findings were validated in an external cohort from a secondary referral institution (IPF n=34; FHP n=28).ResultsLow-density lobules and air trapping were a frequent finding in IPF, present in up to 51% of patients. A requirement for increasing extent of low-density lobules and air trapping based on guidelines (American Thoracic Society and Fleischner Society) was associated with increased specificity for the diagnosis of FHP (0.96 and 0.98, respectively) but reduced sensitivity (0.16 and 0.20, respectively). The headcheese sign was found to be highly specific (0.93) and moderately sensitive (0.49) for a high-confidence diagnosis of FHP. The high specificity of the headcheese sign was maintained in the validation cohort and when patients with other CT features of FHP were excluded.ConclusionMosaic attenuation is a frequent finding in IPF. However, the headcheese sign can be confidently considered as being inconsistent with a diagnosis of IPF and specific for FHP.

Living with idiopathic pulmonary fibrosis

2011 ◽  
Vol 8 (4) ◽  
pp. 225-231 ◽  
Author(s):  
Gadi Schoenheit ◽  
Ian Becattelli ◽  
Alan H Cohen
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Medical Care ◽  
Treatment Options ◽  
Unknown Origin ◽  
Well Being ◽  
Accurate Diagnosis ◽  
Initial Presentation ◽  
Diagnostic Process ◽  
Centers Of Excellence

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fatal lung disease of unknown origin. Despite recent advancements, the diagnosis and management of IPF remains a distinct clinical challenge; as a result, patients often experience considerable delays in receiving an accurate diagnosis and confusion regarding prognosis and the availability of treatment options. In order to gain further insights regarding patients' perspectives on the diagnostic process, disease education, emotional well-being, and quality of life, we conducted a qualitative in-depth survey among IPF patients in Europe. Patients with a physician-confirmed IPF diagnosis were recruited to participate in an in-depth interview conducted by a trained facilitator who used a qualitative topic guide. A total of 45 patients from 5 European countries participated in the survey. The median reported time from initial presentation to confirmed diagnosis of IPF was 1.5 years (range <1 week to 12 years); in 58% of cases there was a delay of >1 year between initial presentation and a confirmed diagnosis of IPF. Additionally, 55% of patients reported consulting ≥3 physicians before receiving an IPF diagnosis. Patient satisfaction with medical care and disease education appeared to be higher among patients who were receiving care at a recognized center of excellence. Patients generally had reasonable expectations regarding the goals of therapy, with most recognizing the irreversibility of the disease and the limited prospects for achieving a cure. The most common unmet needs cited by participants were disease education resources, access to centers of excellence, and familial support programs. Our findings suggest that patients with IPF commonly experience protracted delays in receiving an accurate diagnosis and generally perceive the level of medical care as suboptimal, despite expressing reasonable expectations regarding the goals of therapy. These results support the need for further improvement in the areas of diagnosis, disease management, and patient education.

scholarly journals Making an Accurate Diagnosis of Chronic Hypersensitivity Pneumonitis

2014 ◽  
Vol 21 (6) ◽  
pp. 370-372 ◽  
Author(s):  
Kerri A Johannson ◽  
Christopher J Ryerson
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Present Article ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Hypersensitivity Pneumonitis ◽  
Interstitial Lung Diseases ◽  
Accurate Diagnosis ◽  
Management Approach ◽  
Chronic Hypersensitivity Pneumonitis

Chronic hypersensitivity pneumonitis (HP) arises from repeated exposure to causative antigens. Although HP can be challenging to diagnose, it is important to differentiate from idiopathic pulmonary fibrosis and idiopathic non-specific interstitial pneumonia. HP has a unique management approach and portends a unique prognosis. The present article summarizes the recent published literature on chronic HP and highlights the features that may be helpful in distinguishing it from other chronic interstitial lung diseases.

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