scholarly journals Idiopathic Interstitial Pneumonias: a Unifying Concept

Doctor Ru ◽  
2018 ◽  
Vol 148 (4) ◽  
pp. 14-17
Author(s):  
M.M. Ilkovich ◽  
◽  
L.N. Novikova ◽  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Early Diagnosis ◽  
Lung Disease ◽  
Cryptogenic Organizing Pneumonia ◽  
Idiopathic Interstitial Pneumonias ◽  
Systemic Corticosteroids ◽  
Principles Of Treatment ◽  
Antifibrotic Agents ◽  
Fibrotic Lung Disease

Idiopathic interstitial pneumonias (IIP) can be combined under the term "idiopathic fibrotic lung disease". It has a number of characteristic clinical and morphological features, which depend on the course of the disease (acute, subacute, or chronic) and are never identical in the early and end stages. Combining all types of IIP, other than unclassified interstitial pneumonias and cryptogenic organizing pneumonia, under the term "idiopathic fibrotic lung disease" will make it possible to move away from the ambiguous concept of so-called early diagnosis of idiopathic pulmonary fibrosis, establish criteria of disease activity, and identify clear indications for systemic corticosteroids and new antifibrotic agents in patients with IIP. Keywords: idiopathic interstitial pneumonias, idiopathic pulmonary fibrosis, early diagnosis, principles of treatment.

scholarly journals Complications in Idiopathic Pulmonary Fibrosis: Focus on Their Clinical and Radiological Features

Diagnostics ◽  
2020 ◽  
Vol 10 (7) ◽  
pp. 450
Author(s):  
Federica Galioto ◽  
Stefano Palmucci ◽  
Giovanna M. Astuti ◽  
Ada Vancheri ◽  
Giulio Distefano ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Integrated Approach ◽  
Correct Treatment ◽  
Radiological Features ◽  
Palliative Care Services ◽  
Pictorial Essay ◽  
Personalized Approach ◽  
Fibrotic Lung Disease

Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease with uncertain origins and pathogenesis; it represents the most common interstitial lung disease (ILD), associated with a pathological pattern of usual interstitial pneumonitis (UIP). This disease has a poor prognosis, having the most lethal prognosis among ILDs. In fact, the progressive fibrosis related to IPF could lead to the development of complications, such as acute exacerbation, lung cancer, infections, pneumothorax and pulmonary hypertension. Pneumologists, radiologists and pathologists play a key role in the identification of IPF disease, and in the characterization of its complications—which unfortunately increase disease mortality and reduce overall survival. The early identification of these complications is very important, and requires an integrated approach among specialists, in order to plane the correct treatment. In some cases, the degree of severity of patients having IPF complications may require a personalized approach, based on palliative care services. Therefore, in this paper, we have focused on clinical and radiological features of the complications that occurred in our IPF patients, providing a comprehensive and accurate pictorial essay for clinicians, radiologists and surgeons involved in their management.

scholarly journals The Its Not JUST Idiopathic pulmonary fibrosis Study (INJUSTIS): description of the protocol for a multicentre prospective observational cohort study identifying biomarkers of progressive fibrotic lung disease

2019 ◽  
Vol 6 (1) ◽  
pp. e000439 ◽  
Author(s):  
Fasihul Khan ◽  
Iain Stewart ◽  
Lucy Howard ◽  
Tricia M McKeever ◽  
Steve Jones ◽  
...  
Keyword(s):  
Cohort Study ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Molecular Mechanisms ◽  
Observational Cohort Study ◽  
Prospective Observational Cohort Study ◽  
Ethical Approval ◽  
Observational Cohort ◽  
Fibrotic Lung Disease

IntroductionThe Its Not JUST Idiopathic pulmonary fibrosis Study (INJUSTIS) is a multicentre, prospective, observational cohort study. The aims of this study are to identify genetic, serum and other biomarkers that may identify specific molecular mechanisms, reflecting disease endotypes that are shared among patients with progressive pulmonary fibrosis regardless of aetiology. Furthermore, it is anticipated that these biomarkers will help predict fibrotic activity that may identify patterns of disease behaviour with greater accuracy than current clinical phenotyping.Methods and analysis200 participants with the multidisciplinary team confirmed fibrotic lung disease (50 each of rheumatoid-interstitial lung disease (ILD), asbestosis, chronic hypersensitivity pneumonitis and unclassifiable ILD) and 50 idiopathic pulmonary fibrosis participants, recruited as positive controls, will be followed up for 2 years. Participants will have blood samples, lung function tests, quality of life questionnaires and a subgroup will be offered bronchoscopy. Participants will also be given the option of undertaking blinded home handheld spirometry for the first 3 months of the study. The primary end point will be identification of a biomarker that predicts disease progression, defined as 10% relative change in forced vital capacity (FVC) or death at 12 months.Ethics and disseminationThe trial has received ethical approval from the National Research Ethics Committee Nottingham (18/EM/0139). All participants must provide written informed consent. The trial will be overseen by the INJUSTIS steering group that will include a patient representative, and an independent chairperson. The results from this study will be submitted for publication in peer-reviewed journals and disseminated at regional and national conferences.Trial registration numberNCT03670576.

scholarly journals Idiopathic pulmonary fibrosis: the radiologist’s role in making the diagnosis

2019 ◽  
Vol 92 (1099) ◽  
pp. 20181003 ◽  
Author(s):  
Michael P. Mohning ◽  
John Caleb Richards ◽  
Tristan J. Huie
Keyword(s):  
Differential Diagnosis ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Lung Biopsy ◽  
Critical Role ◽  
Pattern Identification ◽  
Diagnostic Process ◽  
Imaging Features ◽  
Fibrotic Lung Disease

Radiologists have a critical role in the evaluation and diagnosis of suspected idiopathic pulmonary fibrosis (IPF). Accurate pattern identification on imaging is key in the multidisciplinary diagnostic process and frequently obviates the need for a surgical lung biopsy. In this review, we describe the clinical and imaging features of IPF in the context of recently revised international guidelines; contrast findings in other diseases that may inform differential diagnosis of fibrotic lung disease; and highlight common complications associated with pulmonary fibrosis.

scholarly journals Deep learning in interstitial lung disease—how long until daily practice

2020 ◽  
Vol 30 (11) ◽  
pp. 6285-6292
Author(s):  
Ana Adriana Trusculescu ◽  
Diana Manolescu ◽  
Emanuela Tudorache ◽  
Cristian Oancea
Keyword(s):  
Deep Learning ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Early Diagnosis ◽  
Lung Disease ◽  
Lung Diseases ◽  
Daily Practice ◽  
Interstitial Lung Diseases ◽  
Learning Approaches ◽  
High Resolution Computed Tomography

Abstract Interstitial lung diseases are a diverse group of disorders that involve inflammation and fibrosis of interstitium, with clinical, radiological, and pathological overlapping features. These are an important cause of morbidity and mortality among lung diseases. This review describes computer-aided diagnosis systems centered on deep learning approaches that improve the diagnostic of interstitial lung diseases. We highlighted the challenges and the implementation of important daily practice, especially in the early diagnosis of idiopathic pulmonary fibrosis (IPF). Developing a convolutional neuronal network (CNN) that could be deployed on any computer station and be accessible to non-academic centers is the next frontier that needs to be crossed. In the future, early diagnosis of IPF should be possible. CNN might not only spare the human resources but also will reduce the costs spent on all the social and healthcare aspects of this deadly disease. Key Points • Deep learning algorithms are used in pattern recognition of different interstitial lung diseases. • High-resolution computed tomography plays a central role in the diagnosis and in the management of all interstitial lung diseases, especially fibrotic lung disease. • Developing an accessible algorithm that could be deployed on any computer station and be used in non-academic centers is the next frontier in the early diagnosis of idiopathic pulmonary fibrosis.

scholarly journals Management of patients with fibrosing interstitial lung diseases

2021 ◽  
Author(s):  
Lee E Morrow ◽  
Daniel Hilleman ◽  
Mark A Malesker
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Lung Diseases ◽  
Underlying Disease ◽  
Interstitial Lung Diseases ◽  
Interstitial Space ◽  
Fda Approval ◽  
Antifibrotic Agents

Abstract Disclaimer In an effort to expedite the publication of articles, AJHP is posting manuscripts online as soon as possible after acceptance. Accepted manuscripts have been peer-reviewed and copyedited, but are posted online before technical formatting and author proofing. These manuscripts are not the final version of record and will be replaced with the final article (formatted per AJHP style and proofed by the authors) at a later time. Purpose This article summarizes the appropriate use and pharmacology of treatments for fibrosing interstitial lung diseases, with a specific focus on the antifibrotic agents nintedanib and pirfenidone. Summary The interstitial lung diseases are a heterogenous group of parenchymal lung disorders with a common feature—infiltration of the interstitial space with derangement of the normal capillary-alveolar anatomy. Diseases characterized by fibrosis of the interstitial space are referred to as the fibrosing interstitial lung diseases and often show progression over time: idiopathic pulmonary fibrosis is the most common fibrotic interstitial lung disease. Historically, therapies for fibrosing lung diseases have been limited in number, questionable in efficacy, and associated with potential harms. Food and Drug Administration (FDA) approval of the antifibrotic agents nintedanib and pirfenidone for idiopathic pulmonary fibrosis in 2014 heralded an era of reorganization of therapy for the fibrotic interstitial lung diseases. Subsequent investigations have led to FDA approval of nintedanib for systemic sclerosis–associated interstitial lung disease and interstitial lung diseases with a progressive phenotype. Although supportive care and pulmonary rehabilitation should be provided to all patients, the role(s) of immunomodulators and/or immune suppressing agents vary by the underlying disease state. Several agents previously used to treat fibrotic lung diseases (N-acetylcysteine, anticoagulation, pulmonary vasodilators) lack efficacy or cause harm. Conclusion With the introduction of effective pharmacotherapy for fibrosing interstitial lung disease, pharmacists have an increasingly important role in the interdisciplinary team managing these patients.

Real-World Comprehensive Disease Management of Patients With Idiopathic Pulmonary Fibrosis

2019 ◽  
Vol 15 (1) ◽  
pp. 4-15 ◽  
Author(s):  
Wendi Mason ◽  
Sally McLaughlin ◽  
Sophy Dedopoulos ◽  
Erin Mahoney ◽  
Tonja Meadows ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Disease Progression ◽  
Pulmonary Rehabilitation ◽  
Poor Prognosis ◽  
Antifibrotic Therapy ◽  
Fibrotic Lung Disease ◽  
Long Term Oxygen Therapy

Idiopathic pulmonary fibrosis (IPF) is a debilitating, progressive, and fatal fibrotic lung disease with a poor prognosis. Antifibrotic therapy slows but does not halt disease progression. Patient education and management needs change during disease progression. Management is complicated by comorbidities, adverse events associated with antifibrotic therapy, and difficulties with long-term oxygen therapy and pulmonary rehabilitation. Treating IPF requires coordination between physicians and nurses in community and interstitial lung disease center settings. This review provides guidance for the healthcare professional who manages the essential aspects of care in IPF from diagnosis, through disease progression, and to the end of life.

scholarly journals The histologic diagnosis of usual interstitial pneumonia of idiopathic pulmonary fibrosis. Where we are and where we need to go

2021 ◽  
Author(s):  
Maxwell L. Smith
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Clinical Syndrome ◽  
Histologic Diagnosis ◽  
Current State ◽  
History Of ◽  
Fibrotic Lung Disease

AbstractIn the 50 years since its inception by Dr. Liebow, the diagnosis of usual interstitial pneumonia (UIP) by pathologists has changed significantly. This manuscript reviews the progressive history of the histologic diagnosis of UIP and summarizes the current state of histologic UIP and its relationship to the clinical syndrome idiopathic pulmonary fibrosis (IPF). Fibrotic lung disease mimics of UIP/IPF are reviewed and pearls for distinguishing these diseases from UIP/IPF are provided. Strategies for increasing the value of histologic assessment of biopsies in the setting of pulmonary fibrosis are also discussed.

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