scholarly journals Antifibrotic Therapies and Progressive Fibrosing Interstitial Lung Disease (PF-ILD): Building on INBUILD

2021 ◽  
Vol 10 (11) ◽  
pp. 2285
Author(s):  
John N. Shumar ◽  
Abhimanyu Chandel ◽  
Christopher S. King
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Treatment Pathway ◽  
Antifibrotic Therapy ◽  
New Treatment ◽  
Fibrotic Lung Diseases

Progressive fibrosing interstitial lung disease (PF-ILD) describes a phenotypic subset of interstitial lung diseases characterized by progressive, intractable lung fibrosis. PF-ILD is separate from, but has radiographic, histopathologic, and clinical similarities to idiopathic pulmonary fibrosis. Two antifibrotic medications, nintedanib and pirfenidone, have been approved for use in patients with idiopathic pulmonary fibrosis. Recently completed randomized controlled trials have demonstrated the clinical efficacy of antifibrotic therapy in patients with PF-ILD. The validation of efficacy of antifibrotic therapy in PF-ILD has changed the treatment landscape for all of the fibrotic lung diseases, providing a new treatment pathway and opening the door for combined antifibrotic and immunosuppressant drug therapy to address both the fibrotic and inflammatory components of ILD characterized by mixed pathophysiologic pathways.

Chronic fibrosing progressing interstitial lung disease: a decision of Multidisciplinary Expert Board

2021 ◽  
Vol 31 (4) ◽  
pp. 505-510
Author(s):  
S. N. Avdeev ◽  
S. Yu. Chikina ◽  
I. E. Tyurin ◽  
A. S. Belevskiy ◽  
S. A. Terpigorev ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Russian Federation ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Diagnosis And Treatment ◽  
Antifibrotic Therapy

Introduction. The natural course of some interstitial lung diseases (ILD) is characterized by progressive fibrosing phenotype resembling idiopathic pulmonary fibrosis (IPF). Until recently, the antifibrotic drug nintedanib was approved for treatment of the only fibrosing ILD which was IPF. A new indication for this drug which has been registered in Russian Federation in 2021 includes other fibrosing ILDs with progressive phenotype (PF-ILDs) and ILD associated with systemic scleroderma (SS-ILD).The aim of this publication is to describe general considerations of the decision of Multidisciplinary Expert Board on diagnosis and treatment of PF-ILDs including SS-ILD.Results. According to the extension in nintedanib use mentioned above, the Expert Board created an algorithm for diagnosis and treatment of patients with PF-ILDs and criteria for nuntedanib administration in PF-ILDs.Conclusion. Antifibrotic therapy is needed for patients with PF-ILDs with the failure of the stanrard therapy. In those patients antifibrotic treatment should be initiated as early as possible to better preserve the lung function.

scholarly journals Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis

2019 ◽  
Vol 28 (153) ◽  
pp. 190022 ◽  
Author(s):  
Bridget F. Collins ◽  
Ganesh Raghu
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Lung Diseases ◽  
Standard Of Care ◽  
Interstitial Lung Diseases ◽  
High Resolution Computed Tomography ◽  
Pharmacological Agents ◽  
Antifibrotic Therapy ◽  
Fibrotic Lung Diseases

Two antifibrotic medications (nintedanib and pirfenidone) were recommended (conditionally) for the treatment of patients with idiopathic pulmonary fibrosis (IPF) in the 2015 IPF evidence-based guidelines. These medications have been shown to reduce the rate of decline in forced vital capacity among patients with IPF over time and are the only two disease-modulating pharmacological agents approved by regulatory agencies and available for clinical use worldwide. With the evolved standard of care for interstitial lung disease evaluation including routine use of high-resolution computed tomography, fibrotic lung diseases other than IPF are increasingly recognised. In addition, it is becoming evident that genetic and pathophysiological mechanisms as well as disease behaviour in patients manifesting other “non-IPF progressive fibrotic interstitial lung diseases” (non-IPF-PF) may be similar to those in patients with IPF. Thus, it is biologically plausible that pharmacological agents with antifibrotic properties may be efficacious in non-IPF-PF. Indeed, studies are underway or planned to assess the safety and efficacy of nintedanib or pirfenidone among patients with several non-IPF fibrotic lung diseases. In this review, we briefly summarise the use of pirfenidone and nintedanib in IPF as well as the rationale and potential for use of these medications in non-IPF-PF that are being investigated in ongoing and upcoming clinical trials.

scholarly journals Comparison of Characteristics of Connective Tissue Disease-Associated Interstitial Lung Diseases, Undifferentiated Connective Tissue Disease-Associated Interstitial Lung Diseases, and Idiopathic Pulmonary Fibrosis in Chinese Han Population: A Retrospective Study

2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Lin Pan ◽  
Yuan Liu ◽  
Rongfei Sun ◽  
Mingyu Fan ◽  
Guixiu Shi
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Western China ◽  
Undifferentiated Connective Tissue Disease

Our study compared the prevalence and characteristics of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD), undifferentiated connective tissue disease-associated interstitial lung disease (UCTD-ILD), or idiopathic pulmonary fibrosis (IPF) between January 2009 and December 2012 in West China Hospital, western China. Patients who met the criteria for ILD were included and were assigned to CTD-ILD, UCTD-ILD, or IPF group when they met the criteria for CTD, UCTD, or IPF, respectively. Clinical characteristics, laboratory tests, and high-resolution CT images were analyzed and compared among three groups. 203 patients were included, and all were Han nationality. CTD-ILD was identified in 31%, UCTD-ILD in 32%, and IPF in 37%. Gender and age differed among groups. Pulmonary symptoms were more common in IPF, while extrapulmonary symptoms were more common in CTD-ILD and UCTD-ILD group. Patients with CTD-ILD had more abnormal antibody tests than those of UCTD-ILD and IPF. Little significance was seen in HRCT images among three groups. A systematic evaluation of symptoms and serologic tests in patients with ILD can identify CTD-ILD, UCTD-ILD, and IPF.

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.

Idiopathic pulmonary fibrosis

2017 ◽  
Author(s):  
Clare Ross ◽  
Athol Wells
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Heterogeneous Group

Interstitial lung diseases are a complex heterogeneous group which are challenging to diagnose and treat. The diagnosis of idiopathic pulmonary fibrosis, as opposed to connective tissue interstitial lung disease, is important, as treatments and prognoses are very different. This chapter focusses on a case where this diagnosis is challenging. The evidence for the treatment of idiopathic pulmonary fibrosis is reviewed, in light of recent trials and new recommendations.

scholarly journals Differential diagnosis of idiopathic pulmonary fibrosis

2020 ◽  
Vol 92 (3) ◽  
pp. 102-108
Author(s):  
E. I. Shmelev ◽  
A. E. Ergeshov ◽  
V. Ya. Gergert
Keyword(s):  
Differential Diagnosis ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Diseases ◽  
Respiratory Diseases ◽  
Clinical Signs ◽  
Interstitial Lung Diseases ◽  
Antifibrotic Therapy ◽  
Inflammatory Drugs ◽  
Diffuse Lung

The review is devoted to the urgent problem of modern pulmonology: the differential diagnosis of idiopathic pulmonary fibrosis (ILF). ILF occupies a special place among many interstitial lung diseases for a number of reasons: 1) it is a deadly disease; 2) early diagnosis and adequate antifibrotic therapy significantly extend the life expectancy of patients; 3) anti-inflammatory drugs (corticosteroids) and cytostatics with ILF that are widely used in other forms of interstitial lung diseases are ineffective and accelerate the progression of the process; 4) the commonality of the main clinical signs (increasing respiratory failure) of various interstitial lung diseases. The list of respiratory diseases with which ILF should be differentiated is huge, and if with diffuse lung lesions of a known nature (disseminated pulmonary tuberculosis, pneumoconiosis, etc.) with a certain experience/qualification, the diagnosis is relatively simple, then the isolation of ILF from the group of idiopathic interstitial pneumonias always represents certain difficulties. The main methods used in the diagnosis of ILF are summarized taking into account current international and national recommendations.

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.

Interstitial lung disease

2007 ◽  
pp. 221-226
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Usual Interstitial Pneumonia ◽  
Interstitial Lung Diseases ◽  
Drug Induced ◽  
Cryptogenic Fibrosing Alveolitis ◽  
Disease Entities

Introduction 222 Known idiopathic pulmonary fibrosis 222 Other idiopathic interstitial lung diseases 223 Drug-induced interstitial lung disease 224 Hypersensitivity pneumonitis (HP) 224 The classification of interstitial lung disease (ILD) has been refined significantly over recent years and is rather confusing to the uninitiated! Most ILDs are rare and unlikely to present as an emergency. Cryptogenic fibrosing alveolitis (CFA), also known as idiopathic pulmonary fibrosis (IPF), is probably the most frequent ILD encountered in routine respiratory practice. The pathology underlying this is termed ‘usual interstitial pneumonia’ (UIP) and it is one of the so-called ‘idiopathic interstitial pneumonias’ (IIPs). These three terms (IPF, CFA, UIP) are often used interchangeably in the same patient's notes which can easily cause further confusion! Description of the pathological distinction between specific disease entities is beyond the scope of this chapter (see OHRM, Chapter 35)....

scholarly journals ABCA 3 mutation associated childhood Interstitial Lung Disease (chILD) presenting as Combined Pulmonary Fibrosis and Emphysema in siblings.

2020 ◽  
Author(s):  
Manjit Tendolkar ◽  
Rahul Tyagi ◽  
Ajay Handa
Keyword(s):  
Interstitial Lung Disease ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Rare Presentation ◽  
Childhood Interstitial Lung Disease

Childhood interstitial lung diseases (chILD) are a rare presentation. We report case of siblings diagnosed with combined pulmonary fibrosis and emphysema (CPFE) attributable to ABCA 3 mutation. It highlights the progressive nature of the disease as it unfolds itself in both the siblings at around the same age. This is the second case of chILD with CPFE reported in literature and the first evidence of its occurrence in siblings adds to the novelty.

Sign in / Sign up

Export Citation Format

Share Document