scholarly journals S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis

Respiration ◽  
2021 ◽  
pp. 1-34
Author(s):  
Jürgen Behr ◽  
Andreas Günther ◽  
Francesco Bonella ◽  
Julien Dinkel ◽  
Ludger Fink ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Diagnosis ◽  
Diagnostic Process ◽  
High Resolution Computed Tomography ◽  
Cellular Analysis ◽  
Golden Standard ◽  
Technical Advances ◽  
Transbronchial Lung Cryobiopsy ◽  
Effective Diagnosis

Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease. Diagnosis of IPF requires considerable expertise and experience. Since the publication of the international IPF guideline in the year 2011 and the update 2018 several studies and technical advances have occurred, which made a new assessment of the diagnostic process mandatory. The goal of this guideline is to foster early, confident, and effective diagnosis of IPF. The guideline focusses on the typical clinical context of an IPF patient and provides tools to exclude known causes of interstitial lung disease including standardized questionnaires, serologic testing, and cellular analysis of bronchoalveolar lavage. High-resolution computed tomography remains crucial in the diagnostic workup. If it is necessary to obtain specimens for histology, transbronchial lung cryobiopsy is the primary approach, while surgical lung biopsy is reserved for patients who are fit for it and in whom a bronchoscopic diagnosis did not provide the information needed. After all, IPF is a diagnosis of exclusion and multidisciplinary discussion remains the golden standard of diagnosis.

scholarly journals Diagnostic Disparity of Previous and Revised American Thoracic Society Guidelines for Idiopathic Pulmonary Fibrosis

2015 ◽  
Vol 22 (2) ◽  
pp. 86-90 ◽  
Author(s):  
Lee Fidler ◽  
Shane Shapera ◽  
Shikha Mittoo ◽  
Theodore K Marras
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Diagnostic Criteria ◽  
Retrospective Chart Review ◽  
Patient Characteristics ◽  
Disease Diagnosis ◽  
American Thoracic Society ◽  
Patient Classification ◽  
High Resolution Computed Tomography ◽  
The Impact

BACKGROUND: A revised guideline for the diagnosis of idiopathic pulmonary fibrosis (IPF) was formulated by the American Thoracic Society (ATS) in 2011 to improve disease diagnosis and provide a simplified algorithm for clinicians. The impact of these revisions on patient classification, however, remain unclear.OBJECTIVE: To examine the concordance between diagnostic guidelines to understand how revisions impact patient classification.METHODS: A cohort of 54 patients with either suspected IPF or a working diagnosis of IPF was evaluated in a retrospective chart review, in which patient data were examined according to previous and revised ATS guidelines. Patient characteristics influencing the fulfillment of diagnostic criteria were compared using one-way ANOVA and χ2tests.RESULTS: Revised and previous guideline criteria for IPF were met in 78% and 83% of patients, respectively. Revised guidelines modified a classification based on previous guidelines in 28% of cases. Fifteen percent of patients meeting previous ATS guidelines failed to meet revised criteria due to a lack of honeycombing on high-resolution computed tomography and the absence of a surgical lung biopsy. Patients failing to meet previous and revised diagnostic criteria for IPF were younger.CONCLUSION: The revised guidelines for the diagnosis of IPF classify a substantial proportion of patients differently than the previous guidelines.

scholarly journals Acute Exacerbation of Idiopathic Pulmonary Fibrosis

2018 ◽  
Author(s):  
Tomoo Kishaba
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Acute Exacerbation ◽  
Stable State ◽  
Volume Overload ◽  
Clinical History ◽  
Diagnostic Process ◽  
High Resolution Computed Tomography ◽  
Laboratory Findings ◽  
Progressive Respiratory Failure

Idiopathic Pulmonary Fibrosis (IPF) is most common idiopathic interstitial pneumonias. IIPF is often seen in elderly smoker man. Diagnosis of IPF is integration of detailed clinical history, specific physical examination, laboratory findings, pulmonary function test, chest high-resolution computed tomography (HRCT) and pathology. IPF have heterogeneous clinical course from asymptomatic stable state to progressive respiratory failure or acute exacerbation (AE). AE of IPF have several important differential diagnosis such as heart failure and volume overload. International working project proposed new criteria of AE of IPF in 2016. They divided into triggered and idiopathic AE. On the basis of this criteria, physician can capture AE of IPF more easily. Recent international IPF guideline emphasized the utility of chest HRCT. In addition, two anti-fibrotic agents have been available. We should pay attention to not only management of AE, but also prevention it. I review diagnostic process, laboratory findings, typical chest imaging, management and prognosis of AE.

scholarly journals Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Medicina ◽  
2019 ◽  
Vol 55 (3) ◽  
pp. 70 ◽  
Author(s):  
Tomoo Kishaba
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Acute Exacerbation ◽  
Pulmonary Function Tests ◽  
Stable State ◽  
Clinical History ◽  
Diagnostic Process ◽  
Group Project ◽  
High Resolution Computed Tomography ◽  
Laboratory Findings

Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonia. Idiopathic pulmonary fibrosis is often seen in elderly men who smoke. A diagnosis of IPF is based on a combination of a detailed clinical history, specific physical examination, laboratory findings, pulmonary function tests, high-resolution computed tomography (HRCT) of the chest, and histopathology. Idiopathic pulmonary fibrosis has a heterogeneous clinical course, from an asymptomatic stable state to progressive respiratory failure or acute exacerbation (AE). Acute exacerbation of IPF has several important differential diagnoses, such as heart failure and volume overload. The International Working Group project proposed new criteria for defining AE of IPF in 2016, which divides it into triggered and idiopathic AE. On the basis of these criteria, physicians can detect AE of IPF more easily. The recent international IPF guidelines emphasized the utility of chest HRCT. In addition, two antifibrotic agents have become available. We should focus on both the management and prevention of AE. The diagnostic process, laboratory findings, typical chest imaging, management, and prognosis of AE are comprehensively reviewed in this article.

scholarly journals Performance of Radiomics Features in the Quantification of Idiopathic Pulmonary Fibrosis from HRCT

Diagnostics ◽  
2020 ◽  
Vol 10 (5) ◽  
pp. 306 ◽  
Author(s):  
Alessandro Stefano ◽  
Mauro Gioè ◽  
Giorgio Russo ◽  
Stefano Palmucci ◽  
Sebastiano Emanuele Torrisi ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Hounsfield Unit ◽  
Poisson Approximation ◽  
Diagnostic Value ◽  
Diagnostic Process ◽  
High Resolution Computed Tomography ◽  
The Relationship

Background: Our study assesses the diagnostic value of different features extracted from high resolution computed tomography (HRCT) images of patients with idiopathic pulmonary fibrosis. These features are investigated over a range of HRCT lung volume measurements (in Hounsfield Units) for which no prior study has yet been published. In particular, we provide a comparison of their diagnostic value at different Hounsfield Unit (HU) thresholds, including corresponding pulmonary functional tests. Methods: We consider thirty-two patients retrospectively for whom both HRCT examinations and spirometry tests were available. First, we analyse the HRCT histogram to extract quantitative lung fibrosis features. Next, we evaluate the relationship between pulmonary function and the HRCT features at selected HU thresholds, namely −200 HU, 0 HU, and +200 HU. We model the relationship using a Poisson approximation to identify the measure with the highest log-likelihood. Results: Our Poisson models reveal no difference at the −200 and 0 HU thresholds. However, inferential conclusions change at the +200 HU threshold. Among the HRCT features considered, the percentage of normally attenuated lung at −200 HU shows the most significant diagnostic utility. Conclusions: The percentage of normally attenuated lung can be used together with qualitative HRCT assessment and pulmonary function tests to enhance the idiopathic pulmonary fibrosis (IPF) diagnostic process.

scholarly journals Acute Exacerbation of Idiopathic Pulmonary Fibrosis

2018 ◽  
Author(s):  
Tomoo Kishaba
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Acute Exacerbation ◽  
Pulmonary Function Tests ◽  
Stable State ◽  
Volume Overload ◽  
Clinical History ◽  
Diagnostic Process ◽  
High Resolution Computed Tomography ◽  
Laboratory Findings

Idiopathic Pulmonary Fibrosis (IPF) is most common idiopathic interstitial pneumonia. IPF is often seen in elderly men who smoke. Diagnosis of IPF requires integration of a detailed clinical history, specific physical examination, laboratory findings, pulmonary function tests, high-resolution computed tomography (HRCT) of the chest, and histopathology. IPF has a heterogeneous clinical course, from an asymptomatic stable state to progressive respiratory failure or acute exacerbation (AE). AE of IPF has several important differential diagnoses, such as heart failure and volume overload. The International working project proposed new criteria of AE of IPF in 2016 dividing it into triggered and idiopathic AE. On the basis of these criteria, physicians can detect AE of IPF more easily. The recent international IPF guideline emphasized the utility of chest HRCT. In addition, two anti-fibrotic agents have become available. We should attend not only to management of AE, but also to its prevention. The diagnostic process, laboratory findings, typical chest imaging, management and prognosis of AE are comprehensively reviewed.

scholarly journals Chemotherapy in idiopathic pulmonary fibrosis and small-cell lung cancer with poor lung function

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xiyue Zhang ◽  
Wei Li ◽  
Chunyan Li ◽  
Jie Zhang ◽  
Zhenzhong Su
Keyword(s):  
Lung Cancer ◽  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Small Cell Lung Cancer ◽  
Cell Lung Cancer ◽  
Small Cell ◽  
Small Cell Lung ◽  
High Resolution Computed Tomography ◽  
Chemotherapeutic Regimen

Abstract Background Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease with unclear pathogenesis. IPF is considered as a risk factor for lung cancer. Compared to other lung cancers, small-cell lung cancer (SCLC) has a lower incidence, but has a more aggressive course. Patients with IPF and SCLC have a lower survival rate, more difficult treatment, and poorer prognosis. Case presentation Case 1 was of a 66-year-old man with IPF for 5 years, who was admitted to our hospital for dyspnea. Case 2 was of a 68-year-old woman, who presented with chest pains, cough, and dyspnea. Both patients had extremely poor lung function. High-resolution computed tomography and pathology revealed that both patients had IPF and SCLC. Chemotherapy comprising nedaplatin (80 mg/m2) and etoposide (100 mg for 5 days) was initiated for both patients. Antifibrotic agents were continued during the chemotherapeutic regimen. Both patients showed improvement in their condition after treatment. Conclusion The favorable outcomes in these 2 cases suggests that chemotherapy is worth considering in the management of patients having SCLC and IPF with poor lung function.

scholarly journals Effects of Nintedanib on Quantitative Lung Fibrosis Score in Idiopathic Pulmonary Fibrosis

2020 ◽  
Vol 14 (1) ◽  
pp. 22-31
Author(s):  
Lisa Lancaster ◽  
Jonathan Goldin ◽  
Matthias Trampisch ◽  
Grace Hyun Kim ◽  
Jonathan Ilowite ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Progression ◽  
Lung Fibrosis ◽  
Open Label ◽  
High Resolution Computed Tomography ◽  
Double Blind ◽  
Phase Iiib ◽  
Exploratory Data ◽  
Relative Change

Background: Nintedanib slows disease progression in patients with Idiopathic Pulmonary Fibrosis (IPF) by reducing decline in Forced Vital Capacity (FVC). The effects of nintedanib on abnormalities on high-resolution computed tomography scans have not been previously studied. Objective: We conducted a Phase IIIb trial to assess the effects of nintedanib on changes in Quantitative Lung Fibrosis (QLF) score and other measures of disease progression in patients with IPF. Methods: 113 patients were randomized 1:1 to receive nintedanib 150 mg bid or placebo double-blind for ≥6 months, followed by open-label nintedanib. The primary endpoint was the relative change from baseline in QLF score (%) at month 6. Analyses were descriptive and exploratory. Results: Adjusted mean relative changes from baseline in QLF score at month 6 were 11.4% in the nintedanib group (n=42) and 14.6% in the placebo group (n=45) (difference 3.2% [95% CI: −9.2, 15.6]). Adjusted mean absolute changes from baseline in QLF score at month 6 were 0.98% and 1.33% in these groups, respectively (difference 0.35% [95% CI: −1.27, 1.96]). Adjusted mean absolute changes from baseline in FVC at month 6 were −14.2 mL and −83.2 mL in the nintedanib (n=54) and placebo (n=54) groups, respectively (difference 69.0 mL [95% CI: −8.7, 146.8]). Conclusion: Exploratory data suggest that in patients with IPF, 6 months’ treatment with nintedanib was associated with a numerically smaller degree of fibrotic change in the lungs and reduced FVC decline versus placebo. These data support previous findings that nintedanib slows the progression of IPF.

scholarly journals Domain Knowledge-Assisted Automatic Diagnosis of Idiopathic Pulmonary Fibrosis (IPF) Using High Resolution Computed Tomography (HRCT) (Student Abstract)

2020 ◽  
Vol 34 (10) ◽  
pp. 13979-13980
Author(s):  
Wenxi Yu ◽  
Hua Zhou ◽  
Jonathan G. Goldin ◽  
Grace Hyun J. Kim
Keyword(s):  
Computed Tomography ◽  
High Resolution ◽  
Optimal Design ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Domain Knowledge ◽  
Population Level ◽  
Design Criterion ◽  
High Resolution Computed Tomography ◽  
Ct Slices

Domain knowledge acquired from pilot studies is important for medical diagnosis. This paper leverages the population-level domain knowledge based on the D-optimal design criterion to judiciously select CT slices that are meaningful for the disease diagnosis task. As an illustrative example, the diagnosis of idiopathic pulmonary fibrosis (IPF) among interstitial lung disease (ILD) patients is used for this work. IPF diagnosis is complicated and is subject to inter-observer variability. We aim to construct a time/memory-efficient IPF diagnosis model using high resolution computed tomography (HRCT) with domain knowledge-assisted data dimension reduction methods. Four two-dimensional convolutional neural network (2D-CNN) architectures (MobileNet, VGG16, ResNet, and DenseNet) are implemented for an automatic diagnosis of IPF among ILD patients. Axial lung CT images are acquired from five multi-center clinical trials, which sum up to 330 IPF patients and 650 non-IPF ILD patients. Model performance is evaluated using five-fold cross-validation. Depending on the model setup, MobileNet achieved satisfactory results with overall sensitivity, specificity, and accuracy greater than 90%. Further evaluation of independent datasets is underway. Based on our knowledge, this is the first work that (1) uses population-level domain knowledge with optimal design criterion in selecting CT slices and (2) focuses on patient-level IPF diagnosis.

Assessment of Lung Cancer Development in Idiopathic Pulmonary Fibrosis Patients Using Quantitative High-Resolution Computed Tomography

2020 ◽  
Vol 35 (2) ◽  
pp. 115-122 ◽  
Author(s):  
Stefano Palmucci ◽  
Sebastiano E. Torrisi ◽  
Daniele Falsaperla ◽  
Alessandro Stefano ◽  
Alfredo G. Torcitto ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Lung Cancer ◽  
High Resolution ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Cancer Development ◽  
High Resolution Computed Tomography
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