Accurate Diagnosis Of Idiopathic Pulmonary Fibrosis Research Articles

Diagnostic Approaches for Idiopathic Pulmonary Fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial pneumonia with a very poor prognosis. Accurate diagnosis of IPF is essential for good outcomes but remains a major medical challenge due to variability in clinical presentation and the shortcomings of existing diagnostic tests. Medical history collection is the first and most important step in the IPF diagnosis process; the clinical probability of IPF is high if the suspected patient is 60 years or older, male, and has a history of cigarette smoking. Systemic assessment for connective tissue disease is essential in the initial evaluation of patients with suspected IPF to identify potential causes of interstitial lung disease (ILD). Radiologic examination using high-resolution computed tomography plays a pivotal role in the evaluation of patients with ILD, and prone and expiratory computed tomography images can be considered. If additional tests such as surgical lung biopsy or transbronchial lung cryobiopsy are needed, transbronchial lung cryobiopsy should be considered as an alternative to surgical lung biopsy in medical centers with experience performing this procedure. Diagnosis through multidisciplinary discussion (MDD) is strongly recommended as MDD has become the cornerstone for diagnosis of IPF, and the scope of MDD has expanded to monitoring of disease progression and suggestion of appropriate treatment options.

Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is the most common fibrosing lung disease. It is associated with a very poor prognosis. Treatments can delay the progression of IPF, so early diagnosis is fundamental. Radiologists play a fundamental role in the evaluation and accurate diagnosis of IPF. Identifying the characteristic patterns of IPF on high-resolution computed tomography (HRCT) is key in the process of multidisciplinary diagnosis, often obviating the need for surgical lung biopsies. This review describes and illustrates the clinical and imaging findings in IPF in the context of the most recent international guidelines, as well as the differential diagnosis and the role of HRCT in follow-up and assessment of complications.

Fibrosis pulmonar idiopática

Idiopathic pulmonary fibrosis (IPF) is the most common fibrosing lung disease. It is associated with a very poor prognosis. Treatments can delay the progression of IPF, so early diagnosis is fundamental. Radiologists play a fundamental role in the evaluation and accurate diagnosis of IPF. Identifying the characteristic patterns of IPF on high-resolution computed tomography (HRCT) is key in the process of multidisciplinary diagnosis, often obviating the need for surgical lung biopsies. This review describes and illustrates the clinical and imaging findings in IPF in the context of the most recent international guidelines, as well as the differential diagnosis and the role of HRCT in follow-up and assessment of complications.

PerFECT 2.0: A Web-Based Platform Designed to Facilitate and Support the Diagnosis of Patients with Idiopathic Pulmonary Fibrosis in Italy.

IntroductionTimely and accurate diagnosis of idiopathic pulmonary fibrosis (IPF) is challenging, requiring specific tests including chest high-resolution computed tomography (HRCT), and limited by access to specialist centres with a multidisciplinary team (MDT). Here we describe PerFECT 2.0, an Italian web-based platform designed to create a network between tertiary centres with an MDT (hubs) and secondary centres (spokes), aiming to facilitate the diagnosis of IPF.MethodsPerFECT 2.0 went live on 1 November 2016. Spoke centres submit anonymised documentation (HRCT images, pathological samples, clinical data) for a second opinion on the potential diagnosis of IPF from a hub centre. HRCT images are quickly uploaded, with patient-identifying information automatically removed. The hub centre views documentation online (no downloads allowed), makes any further information requests, then returns their second opinion as free text. An e-learning area contains educational material and simulated training clinical cases. Metrics were collected for 2017–2019; a user survey was conducted from 30 June–31 July 2020.ResultsTen hub centres and 137 spoke centres have registered. The requests for a second opinion numbered 251 in 2017, 270 in 2018 and 265 in 2019 (overall mean 19.9 requests per month). The proportion of requests answered was 100.0% (251) in 2017, 100.0% (270) in 2018 and 97.7% (259) in 2019. The mean response time was 15.7 days. In the user survey, of nine hub responders and 19 spoke responders, 78% and 74%, respectively, reported that the platform is easy to use, and 100% and 89%, respectively, would recommend the platform to colleagues.ConclusionThe PerFECT 2.0 web-based platform has created a network that enables secondary centres to gain quick and easy access to a second opinion from a tertiary centre with an MDT through online evaluation of anonymised documentation, thereby facilitating and supporting the timely and accurate diagnosis of IPF.Supplementary InformationThe online version contains supplementary material available at 10.1007/s41030-021-00155-8.

Outcomes of Video-Assisted Thoracic Surgical Lung Biopsy for Interstitial Lung Diseases.

Purpose: Surgical lung biopsy (SLB) is an important diagnostic tool for interstitial lung disease (ILD), yet the risk factors for SLB are still debatable and long-term outcomes remain unknown.Methods: We retrospectively reviewed the records of 85 consecutive patients with ILD who underwent SLB by video-assisted thoracic surgery (VATS) from 2008 to 2019. Risk factors for complications and differences of outcomes between idiopathic pulmonary fibrosis (IPF) and other ILDs were examined.Results: All patients who underwent VATS had no mortality or acute exacerbation of ILD within 90 days of SLB. The rate of complication was 9.4%, and there were no statistically significant risk factors for complications. While the IPF group was not significantly different from the non-IPF group with regard to surgical parameters or complications, patients with IPF had significantly higher rates of mortality (50% vs. 9% in 5 years; p <0.001) and readmission due to acute exacerbation (75% vs. 8% in 5 years; p <0.001).Conclusion: VATS lung biopsy for ILD can be a safe approach regardless of underlying phenotypes. An accurate diagnosis of IPF via SLB may be beneficial for correct patient management.

DIAGNOSING IDIOPATHIC PULMONARY FIBROSIS IN COMMUNITY-BASED PULMONOLARY PRACTICES: FACTORS INFLUENCING APPROACH TO DIAGNOSIS

SESSION TITLE: Diffuse Lung Disease Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: October 18-21, 2020 PURPOSE: Despite the increased awareness of Idiopathic Pulmonary Fibrosis (IPF) and refinement of the guidelines, there is still a significant delay in the diagnosis of IPF as many of these patients are cared for at non-Interstitial Lung Disease Centers of Excellence (ILD-CE). The purpose of this study is to gain further insight into the factors influencing the pulmonologists’ approach to diagnosing IPF at non-ILD-CE. METHODS: This study was approved by the Loma Linda University Institutional Review Board. An anonymous survey was emailed to pulmonologists throughout the United States using selected proprietary medical databases. Pulmonologists practicing at an ILD-CE were excluded. The survey evaluated the pulmonologist's approach related to referral to an ILD-CE, utilization of a Multi-Disciplinary Approach (MDA), and their attitude towards the importance of obtaining an early and accurate diagnosis. RESULTS: There were a total of 58 survey respondents: 35% in hospital-based practices and 65% in community-based practices. Only 55% of participants strongly agreed that obtaining an early and accurate diagnosis of IPF was important; no difference was noted between the practice settings. The median percentage of time the participants used an MDA was 45% (IQR: 10.75-90); 20% in hospital-based practice and 40% in community-based practice (p<0.05). In respondents with access to an ILD-CE, the median percentage of the time patients were referred to one was only 41%. CONCLUSIONS: Based on these preliminary results, the pulmonologist’s perspective on the importance of obtaining an early and accurate IPF diagnosis is low at non-ILD-CE. The utilization of an MDA was also very low despite this being the gold standard for diagnosis for all ILDs as recommended by the American Thoracic Society Task Force. Even with access to an ILD-CE, the referral rate was low. Further studies with higher sample sizes are needed to better understand the rationale behind low utilization rates of MDA and referrals to ILD-CE. CLINICAL IMPLICATIONS: This could reveal the reason behind the large number of patients with IPF that potentially go undiagnosed (or misdiagnosed) in the community-based practices. DISCLOSURES: Stock market shareholder relationship with ABT, ABBV, AZN, BMY, DOW,JNJ, LLY,PFE Please note: $1-$1000 Added 06/09/2020 by Abdullah Alismail, source=Web Response, value=only Quar.Div when applied No relevant relationships by Benjamin Becerra, source=Web Response No relevant relationships by Rodney Biehl, source=Web Response No relevant relationships by Niranjan Jeganathan, source=Web Response No relevant relationships by David Lopez, source=Web Response

Criterios histopatológicos para el diagnóstico de fibrosis pulmonar idiopática

In the latest update of the ATS/ERS/JRS/ALAT Clinical Practice Guidelines for idiopathic pulmonary fibrosis (IPF), a new way of classifying histopathological patterns in 4 types is proposed: definitive usual interstitial pneumonia (UIP), probable UIP, indeterminate and alternative to UIP. A heterogeneous fibrotic remodeling of the normal architecture of the pulmonary parenchyma, with destructive scarring in the form of “honeycomb”, presence of fibroblastic foci and predominantly subpleural and paraseptal distribution, with scarce chronic interstitial inflammatory infiltrate, associated with the absence of elements suggestive of secondary causes such as bronchiolocentric distribution, predominanceof inflammatory interstitial infiltrates or poorly formed granulomas, allows an accurate diagnosis of IPF in an appropriate clinical-radiological scenario.

New Diagnostic Guidelines for Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease characterized by worsening lung function and dyspnea. The prognosis of IPF patients is poor, as median survival is approximately 3 years. However, recently developed IPF-specific therapies have shown improved efficacies in terms of reducing lung function decline and mortality. Therefore, the early recognition and accurate diagnosis of IPF are crucial. In 2018, new guidelines for the diagnosis of IPF were published by the Fleischner Society and by the American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Society (ATS/ERS/JRS/ALAT). Both guidelines emphasize the need for a thorough history taking and physical examination to exclude an alternative diagnosis, such as exposure-related or connective tissue disease. The most informative initial examination is high-resolution computed tomography, the results of which can indicate the need for bronchoalveolar lavage or surgical lung biopsy, based on a multidisciplinary discussion of the findings and the patient’s clinical condition. A multidisciplinary discussion of the clinico-radiologic-pathologic findings is currently the gold standard in the diagnoisis of IPF and will allow the more effective and timely treatment of these patients. Keywords: Idiopathic pulmonary fibrosis; Diagnosis; Guideline 중심 단어: 특발성 íì„¬ìœ ì¦; 진단; 지침

Is It Idiopathic Pulmonary Fibrosis or Not?

Pulmonary fibrosis is not uncommon. Usual interstitial pneumonitis (UIP)/idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic pulmonary fibrotic diseases and has the worst prognosis with a mean life expectancy of 3.8 years. The American Thoracic Society has provided guidelines for the accurate diagnosis of IPF.In 2014, 2 antifibrotic medications were approved in the United States that target the multiple fibrotic pathways of UIP, which increased the need for early and accurate diagnosis of IPF. The early and correct diagnosis is hampered by mimickers that include nonspecific interstitial pneumonitis, chronic hypersensitivity pneumonitis, and fibrotic sarcoidosis. Careful history taking, serologic testing, and Computer Tomography (CT) inspection can frequently make the correct diagnosis without need of invasive procedure. The purpose of this article is to share the most important aspects of the clinical and radiology presentation of IPF and its mimickers to enhance primary care clinician's ability to correctly and noninvasively diagnose UIP/IPF.

Diagnosis of idiopathic pulmonary fibrosis: Current issues.

Idiopathic pulmonary fibrosis (IPF) accounts for the majority of lung diseases classified as idiopathic interstitial pneumonia (IIP). It is considered to be lethal because prognosis is very poor and far worse than other types of IIP. An early and accurate diagnosis of IPF is critical. The diagnostic process is complex and requires a multidisciplinary approach involving a pulmonologist, radiologist and pathologist.

Idiopathic pulmonary fibrosis: current challenges and future perspectives

Idiopathic pulmonary fibrosis (IPF) is the most prevalent of the idiopathic interstitial pneumonias and its incidence seems to be increasing [1–4]. IPF is generally considered to be unresponsive to “standard” therapies and carries a poor prognosis with most patients dying within at least 5 years following diagnosis [5–7]. An accurate diagnosis of IPF is essential for the optimal management of patients with IPF. The original IPF management guidelines, published in 2000 [8], have been superseded recently by the evidence-based international recommendations jointly sponsored by the European Respiratory Society (ERS), the American Thoracic Association (ATS), the Japanese Respiratory Society (JRS) and the Latin American Thoracic Association (ALAT) [9]. Nonetheless, despite this and other major accomplishments over the last decade, the diagnosis and the management of IPF remain a major medical challenge. Diagnostic uncertainties remain due, at least in part, to variability in the natural history of the disease and co-existing complicating comorbidities, interpretation and widespread application of the guideline recommendations, and analysis of …

Managing diagnostic procedures in idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF), the most prevalent of the idiopathic interstitial pneumonias, is associated with a poor prognosis. An accurate diagnosis of IPF is essential for its optimal management. The 2011 American Thoracic Society (ATS)/European Respiratory Society (ERS)/Japanese Respiratory Society (JRS)/Latin American Thoracic Association (ALAT) recommendations on the diagnosis and management of IPF were developed from a systematic review of the published literature. High-resolution computed tomography (HRCT) scanning has a central role in the IPF diagnostic pathway, with formal designation of criteria for an HRCT pattern of usual interstitial pneumonia. In the correct clinical context, a usual interstitial pneumonia pattern on HRCT is indicative of a definite diagnosis of IPF and negates the need for a surgical lung biopsy. However, although the 2011 ATS/ERS/JRS/ALAT statement is a major advance, the application of the guideline recommendations by clinicians has identified limitations that should be addressed in future statements. Key problems include: 1) HRCT misdiagnosis, particularly by less experienced radiologists; 2) lack of management recommendations for the highly prevalent clinical scenarios of "probable" or "possible" IPF; 3) ongoing confusion concerning the diagnostic role of bronchoalveolar lavage; and 4) the lack of integration of clinical data in the designation of the diagnostic likelihood of IPF, including the treated course of disease. These issues become evident as the recommendations are applied and highlight the need for continued guideline adjustments.

The revised ATS/ERS/JRS/ALAT diagnostic criteria for idiopathic pulmonary fibrosis (IPF) - practical implications

Idiopathic pulmonary fibrosis (IPF), the most prevalent idiopathic interstitial pneumonia, is associated with a poor prognosis. An accurate diagnosis of IPF is essential for optimal management. The recent ATS/ERS/JRS/ALAT recommendations on the diagnosis and management of IPF were developed from a systematic review of the published literature. High-resolution computed tomography (HRCT) scanning has a central role in the IPF diagnostic pathway with formal designation of criteria for an HRCT pattern of UIP. In the correct clinical context, a UIP pattern on HRCT is indicative of a definite diagnosis of IPF without the need for a surgical lung biopsy. However, although the 2011 ATS/ERS/JRS/ALAT statement is a major advance, the application of guideline recommendations by clinicians has identified limitations that need to be addressed in future statements. Key problems include: 1) the lack of management recommendations for the highly prevalent clinical scenarios of probable and possible IPF; 2) the ongoing confusion about the diagnostic role of bronchoalveolar lavage (reflecting ambiguity in the current recommendation); 3) HRCT misdiagnosis by less experienced radiologists, increasingly recognised as a major problem; and 4) the lack of integration of clinical data, including the treated course of disease, in the designation of the diagnostic likelihood of IPF.