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

Pulmonary Fibrosis ◽

Lung Disease ◽

Lung Diseases ◽

Childhood Interstitial Lung Disease

Rare Presentation ◽

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.

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

Journal of Clinical Medicine ◽

10.3390/jcm10112285 ◽

2021 ◽

Vol 10 (11) ◽

pp. 2285

Author(s):

John N. Shumar ◽

Abhimanyu Chandel ◽

Christopher S. King

Keyword(s):

Pulmonary Fibrosis ◽

Lung Disease ◽

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.

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

Clinical and Developmental Immunology ◽

10.1155/2013/121578 ◽

2013 ◽

Vol 2013 ◽

pp. 1-7 ◽

Cited By ~ 4

Author(s):

Lin Pan ◽

Yuan Liu ◽

Rongfei Sun ◽

Mingyu Fan ◽

Guixiu Shi

Keyword(s):

Connective Tissue ◽

Pulmonary Fibrosis ◽

Lung Disease ◽

Connective Tissue Disease ◽

Lung Diseases ◽

Undifferentiated Connective Tissue Disease

Western China ◽

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.

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

Russian Pulmonology ◽

10.18093/0869-0189-2021-31-4-505-510 ◽

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):

Lung Function ◽

Pulmonary Fibrosis ◽

Lung Disease ◽

Russian Federation ◽

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.

Management of patients with fibrosing interstitial lung diseases

American Journal of Health-System Pharmacy ◽

10.1093/ajhp/zxab375 ◽

2021 ◽

Author(s):

Lee E Morrow ◽

Daniel Hilleman ◽

Mark A Malesker

Keyword(s):

Pulmonary Fibrosis ◽

Lung Disease ◽

Lung Diseases ◽

Underlying Disease ◽

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

10.1093/med/9780199657742.003.0010 ◽

2017 ◽

Author(s):

Clare Ross ◽

Athol Wells

Keyword(s):

Connective Tissue ◽

Pulmonary Fibrosis ◽

Lung Disease ◽

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.

SAT0304 TOFACITINIB INHIBITS TGF-Β1-INDUCED ACTIVATED CELL FUNCTIONS OF MYOFIBROBLAST IN HUMAN LUNG FIBROBLAST POPULATIONS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.1139 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1097.1-1097

Author(s):

F. Zhu ◽

X. Zhang

Keyword(s):

Lung Disease ◽

Lung Diseases ◽

Human Lung ◽

Migration And Invasion ◽

Control Group ◽

Tgf Β1 ◽

Invasion Assays

Background:Connective tissue disease-associated interstitial lung disease (CTD-ILD) is a class of refractory diseases.Non-specific treatment with hormone and immunosuppressive agents is mostly used at present, but the effect is limited and the long-term survival rate is not improved [1],while anti-fibrosis treatments (such as Pirfenidone and Nintedanib) have only recently been approved, the long-term efficacy is still unknown.Tofacitinib(TOFA), a JAK inhibitor, has recently been used to treat patients with severe dermatomyositis related interstitial pulmonary disease, with significantly improved survival rate [2-4].A basic study showed that TOFA improved interstitial pulmonary disease in mice by promoting the proliferation of myelogenic inhibitory cells [5].However, whether TOFA can affect the migration and invasion of human lung fibroblasts and further research to reveal the mechanism of its inhibition of pulmonary fibrosis has not been reported.Objectives:To investigate the anti - fibrosis effect of TOFA in CTD-ILD.Methods:Cell migration and invasion AssaysHLFs were incubated with TOFA for 72h, followed by TGF- β1 for 24h.DMEM serum-free medium was used to determine the cell density to 5. 0 × 107/L, 600 uL medium containing 10% fetal bovine serum was added to the lower compartment of Transwell chamber, and 200 uL cell suspension was added to the upper compartment.Incubate in incubator for 12 h.After fixation, staining and sealing, the cells were observed and counted under a microscope. At least 5 random field transmembrane cells were counted in each hole, and the mean value was taken.For the invasion assays, Transwell chamber coated with matrigel was used, and the cell incubation time was 16 h.Results:1. Effect of TOFA on HLFs migration function (Figure 1)Figure 1.Effect of TOFA on HLFs migration function(×200).Mean ± SEM. n = 5.The number of cells passing through the biofilm in the three groups was counted.It can be seen that TGF-β1 group significantly increased compared with control group (*P < 0.0001), and TOFA group significantly decreased compared with TGF- β1 group (#P < 0.0001), suggesting that TOFA can significantly inhibit TGF-β1- induced HLFs migration.2. Effect of TOFA on HLFs invasion function (Figure 2)Figure 2.Effect of TOFA on HLFs invasion function(×200).Mean ± SEM. n = 5.The number of cells passing through the matrigel in the three groups was counted.It can be seen that TGF-β1 group was significantly higher than the control group (*P < 0.0001), and TOFA group was significantly lower than TGF-β1 group(#P < 0.001), suggesting that TOFA can significantly inhibit the invasion function of HLFs induced by TGF-β1.Conclusion:TOFA can effectively inhibit the function of HLFs migration and invasion. Although further studies are needed to elucidate the mechanism by which TOFA inhibit the function of HLFs migration and invasion, our study suggests that TOFA has a potential therapeutic effect for CTD-ILD.References:[1]Aparicio, I.J. and J.S. Lee, Connective Tissue Disease-Associated Interstitial Lung Diseases: Unresolved Issues. Semin Respir Crit Care Med, 2016. 37(3): p. 468-76.[2]Kato, M., et al., Successful Treatment for Refractory Interstitial Lung Disease and Pneumomediastinum With Multidisciplinary Therapy Including Tofacitinib in a Patient With Anti-MDA5 Antibody-Positive Dermatomyositis. J Clin Rheumatol, 2019.[3]Kurasawa, K., et al., Tofacitinib for refractory interstitial lung diseases in anti-melanoma differentiation-associated 5 gene antibody-positive dermatomyositis. Rheumatology (Oxford), 2018. 57(12): p. 2114-2119.[4]Chen, Z., X. Wang, and S. Ye, Tofacitinib in Amyopathic Dermatomyositis-Associated Interstitial Lung Disease. N Engl J Med, 2019. 381(3): p. 291-293.[5]Sendo, S., et al., Tofacitinib facilitates the expansion of myeloid-derived suppressor cells and ameliorates interstitial lung disease in SKG mice. Arthritis Res Ther, 2019. 21(1): p. 184Disclosure of Interests:None declared

Interstitial lung disease

10.1093/med/9780199568741.003.0139 ◽

2018 ◽

Author(s):

Patrick Davey ◽

Sherif Gonem ◽

David Sprigings

Keyword(s):

Lung Disease ◽

Lung Diseases ◽

Lung Parenchyma ◽

Broad Group ◽

Diffuse Parenchymal Lung Diseases ◽

Parenchymal Lung

The interstitial lung diseases, also known as the diffuse or diffuse parenchymal lung diseases, are a broad group of pulmonary disorders which mainly affect the lung parenchyma as opposed to the airways. By convention, infectious and malignant conditions are excluded from this definition. Thus, the interstitial lung diseases comprise a group of conditions characterized by variable degrees of inflammation and fibrosis, centred on the lung interstitium and alveolar airspaces.

Radiological diagnosis of interstitial lung diseases

Medicinski pregled ◽

10.2298/mpns13s1029s ◽

2013 ◽

Vol 66 (suppl. 1) ◽

pp. 29-33

Author(s):

Ruza Stevic ◽

Vucinic Mihailovic ◽

Dragana Jovanovic ◽

Nada Vasic

Keyword(s):

Lung Disease ◽

Interstitial Pneumonia ◽

Lung Diseases ◽

Usual Interstitial Pneumonia ◽

Nonspecific Interstitial Pneumonia

Ground Glass ◽

Cryptogenic Organizing Pneumonia ◽

Acute Interstitial Pneumonia ◽

Introduction. Interstitial lung diseases include the entities of idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and lymphocytic interstitial pneumonia. Recent introduction of high-resolution computed tomography has made the diagnosis of interstitial lung disease much easier. Usual interstitial pneumonia A predominantly subpleural reticulation and honeycombing at the basal posterior part of the lung with a progression to anterior and superior parts are characteristic of usual interstitial pneumonia/ idiopathic pulmonary fibrosis. Nonspecific interstitial pneumonia Typical findings of nonspecific interstitial pneumonia are bilateral, relatively symmetrical subpleural ground glass opacifications and irregular linear opacities. Desquamative interstitial pneumonia is characterized by diffuse symmetrical ground glass opacifications. Respiratory bronchiolitisassociated interstitial lung disease Centrilobular nodules and irregular ground glass opacifications are present. Cryptogenic organizing pneumonia Subpleural and peribronchial consolidations are prominent findings that are not present in other idiopathic interstitial pneumonias. Acute interstitial pneumonia. Bilateral ground-glass opacifications are present and areas of peripheral consolidations may also be seen in acute interstitial pneumonia. Lymphocytic interstitial pneumonia. Diffuse or patchy areas of ground glass opacification with centrilobular nodules and occasionally well-defined cysts are seen. Conclusion. Imaging plays a crucial role in identifying interstitial lung diseases but precise diagnosis requires a dynamic interdisciplinary approach that correlates clinical, radiological and pathologic features.

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

European Radiology ◽

10.1007/s00330-020-06986-4 ◽

2020 ◽

Vol 30 (11) ◽

pp. 6285-6292

Author(s):

Ana Adriana Trusculescu ◽

Diana Manolescu ◽

Emanuela Tudorache ◽

Cristian Oancea

Keyword(s):

Deep Learning ◽

Pulmonary Fibrosis ◽

Early Diagnosis ◽

Lung Disease ◽

Lung Diseases ◽

Daily Practice ◽

High Resolution Computed Tomography

Learning Approaches ◽

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.

A contemporary practical approach to the multidisciplinary management of unclassifiable interstitial lung disease

European Respiratory Journal ◽

10.1183/13993003.00276-2021 ◽

2021 ◽

pp. 2100276

Author(s):

Christopher J. Ryerson ◽

Tamera J. Corte ◽

Jeffrey L. Myers ◽

Simon L. F. Walsh ◽

Sabina A. Guler

Keyword(s):

Lung Disease ◽

Lung Diseases ◽

Practical Approach ◽

Multidisciplinary Management ◽

Management Decisions ◽

Histopathological Features ◽

Radiological Features ◽

Immunomodulatory Therapies

Fibrotic interstitial lung diseases (ILDs) frequently have nonspecific and overlapping clinical and radiological features, resulting in approximately 10–20% of patients with ILD lacking a clear diagnosis and thus being labelled with unclassifiable ILD. The objective of this review is to describe how patients with unclassifiable ILD should be evaluated and what impact specific clinical, radiological, and histopathological features may have on management decisions, focusing on patients with a predominantly fibrotic phenotype. We highlight recent data that have suggested an increasing role for antifibrotic medications in a variety of fibrotic ILDs, but justify the ongoing importance of making an accurate ILD diagnosis given the benefit of immunomodulatory therapies in many patient populations. We provide a practical approach to support management decisions that can be used by clinicians and tested by clinical researchers, and further identify the need for additional research to support a rational and standardised approach to the management of patients with unclassifiable ILD.