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Life
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Current Advances in Interstitial Lung Diseases
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Current Advances in Interstitial Lung Diseases

A special issue of Life (ISSN 2075-1729). This special issue belongs to the section "Medical Research".

Deadline for manuscript submissions: closed (25 December 2023) | Viewed by 13348

Special Issue Editors

Prof. Dr. Sergey Avdeev

E-Mail Website
Guest Editor
Pulmonology Department, Sechenov First Moscow State Medical University, 119992 Moscow, Russia
Interests: pulmonary hypertension; idiopathic pulmonary fibrosis; COPD; chronic lung diseases; therapy
Prof. Dr. Sergey V. Moiseev

E-Mail Website
Guest Editor
Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, 119992 Moscow, Russia
Interests: interstitial lung diseases; vasculitis; Fabry disease; eosinophilic granulomatosis with polyangiitis

Special Issue Information

Dear Colleagues,

Interstitial lung disease (ILDs) describes a heterogeneous group of diseases that are classified into subclasses based on similar radiographic or pathologic findings. International guidelines have been developed for the most common forms of ILDs, such as idiopathic pulmonary fibrosis (IPF), sarcoidosis, and hypersensitivity pneumonitis, and are regularly updated to ensure consistency in diagnosis and treatment. However, these well-defined diseases still demonstrate heterogeneity in their prevalence, disease patterns, and outcomes worldwide. Although great advances have been achieved in recent years in understanding ILD pathogeneses and improving the treatment of fibrotic ILD, only a limited part of the world has benefited from these advances. Very few studies have examined the global burden of ILDs or described the variability in their prevalence and subtype between countries. Therefore, studies involving registries of various ILDs (including data from real clinical practice) from different countries are of great interest. Additionally, It should also be noted that today we are witnessing the emergence of a new clinical condition from the circle of ILD, the so-called post-Covid-19 pulmonary fibrosis.

This Special Issue aims to provide up-to-date data by presenting research on ILDs and to provide a comprehensive collection of work performed by experts in the field, thus creating a valuable resource for ILD specialists worldwide. Submissions on the current state of the art as well as ongoing controversies related to the practice of ILDs are welcome.

We are inviting healthcare professionals and researchers involved in ILD patient care to submit original research articles, reviews, or observational studies to Life addressing topics relevant to this issue. Papers will undergo a thorough peer review by experts in the field.

Prof. Dr. Sergey Avdeev
Prof. Dr. Sergey V. Moiseev
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Life is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • interstitial lung diseases
  • idiopathic pulmonary fibrosis
  • hypersensitivity pneumonitis
  • antifibrotic therapy
  • biomarkers

Published Papers (5 papers)

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Research

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11 pages, 803 KiB  
Article
Using Remote Technology to Engage Patients with Interstitial Lung Diseases in a Home Exercise Program: A Pilot Study
by Antonio Sarmento, Kaitlin King and Diana C. Sanchez-Ramirez
Life 2024, 14(2), 265; https://doi.org/10.3390/life14020265 - 17 Feb 2024
Viewed by 970
Abstract
Introduction: The access and compliance of patients with interstitial lung diseases (ILDs) to exercise programs (EPs) remain challenges. Objectives: We assessed the dropout rate, intervention completion, compliance with data acquisition and submission, safety, and satisfaction of a home EP delivered via video conference [...] Read more.
Introduction: The access and compliance of patients with interstitial lung diseases (ILDs) to exercise programs (EPs) remain challenges. Objectives: We assessed the dropout rate, intervention completion, compliance with data acquisition and submission, safety, and satisfaction of a home EP delivered via video conference (EPVC group) or self-directed (EPSD group) to patients with ILD. Pre- and post-intervention changes in patient outcomes (dyspnea, fatigue, exercise capacity, lung function, and quality of life) were secondarily explored. Material and Methods: Groups performed an eight-week virtual EP three times/week. Video conferences were led by a registered respiratory therapist, whereas self-directed exercises were completed following a pre-recorded video. Participants submitted spirometry, heart rate, and SpO2 results weekly to the research team. Results: Fourteen patients with ILD were equally assigned to the EPVC and EPSD groups, but three from the EPSD group dropped out after the initial assessment (dropout rate of 42.8% in the EPSD group). Eleven patients (mean age of 67 ± 12 years) completed 96.5% of sessions. Compliance with data acquisition and submission was optimal (≥97.6% in both groups), and no adverse events were reported. Changes in overall fatigue severity were significantly different between groups (p = 0.014, Cohen’s r = 0.64). Conclusions: The results suggest that a structured virtual EP delivered via video conference or pre-recorded video can be feasible, safe, and acceptable for patients with ILD. Full article
(This article belongs to the Special Issue Current Advances in Interstitial Lung Diseases)
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14 pages, 2405 KiB  
Article
Interstitial Lung Disease in Primary Biliary Cholangitis: A Cohort Prospective Study
by Michail Kalashnikov, Larisa Akulkina, Michail Brovko, Viktoria Sholomova, Alisa Yanakaeva, Dzhamal Abdurakhmanov and Sergey Moiseev
Life 2023, 13(2), 416; https://doi.org/10.3390/life13020416 - 02 Feb 2023
Cited by 1 | Viewed by 2101
Abstract
Interstitial lung disease (ILD) has been recognized as an extrahepatic manifestation ofprimary biliary cholangitis (PBC), althoughlimited data are available on its prevalence and clinical significance. Therefore, we evaluated the occurrence and clinical features of ILD in a cohort of PBC patients. Ninety-three individuals [...] Read more.
Interstitial lung disease (ILD) has been recognized as an extrahepatic manifestation ofprimary biliary cholangitis (PBC), althoughlimited data are available on its prevalence and clinical significance. Therefore, we evaluated the occurrence and clinical features of ILD in a cohort of PBC patients. Ninety-three individuals without concomitant rheumatic diseases were enrolled in our prospective cohort study. All patients underwent chest high-resolution computed tomography (HRCT). Liver-related and lung-related survival wereassessed. A lung-related outcome was defined as death from ILD complications; a liver-related outcome was defined as liver transplantation or death from liver cirrhosis complications. HRCT findings suggestive ofILD were detected in 38 patients (40.9%). A sarcoid-like pattern of PBC-associated ILD was the most frequent, followed by subclinical ILD and organizing pneumonia. Patients with ILD were less likely to have liver cirrhosis and liver-related symptoms and presented with higher serum immunoglobulin M(IgM) and M2 subtype antimitochondrial antibodies (AMA-M2) positivity rates. In a multivariate analysis, the absence of liver disease symptoms at the disease presentation (OR 11.509; 95% CI 1.210–109.421; p = 0.033), the presence of hepatic non-necrotizing epithelioid cell granulomas (OR 17.754; 95% CI 1.805–174.631; p = 0.014), higher serum IgM (OR 1.535; 95% CI 1.067–2.208; p = 0.020) and higher blood leukocyte count (OR 2.356; 95% CI 1.170–4.747; p = 0.016) were independent risk factors associated with ILD in PBC. More than a third of patients with ILD showed no respiratory symptoms, and only one ILD-related death occurred during a follow-up of 29.0 months (IQR 11.5; 38.0). Patients with ILD had better liver transplant-free survival.ILD in PBC had a benign course and was associated with a lower liver disease severity. PBC-associated ILD should be included in a list of differential diagnoses of ILD. Full article
(This article belongs to the Special Issue Current Advances in Interstitial Lung Diseases)
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Review

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17 pages, 342 KiB  
Review
Diagnosis and Pharmacologic Management of Fibrotic Interstitial Lung Disease
by Kristin Berger and Robert J. Kaner
Life 2023, 13(3), 599; https://doi.org/10.3390/life13030599 - 21 Feb 2023
Cited by 3 | Viewed by 2693
Abstract
Interstitial lung disease is an umbrella term that encompasses a spectrum of parenchymal lung pathologies affecting the gas exchanging part of the lung. While many of these disease entities are not fibrotic in nature, a number can lead to pulmonary fibrosis which may [...] Read more.
Interstitial lung disease is an umbrella term that encompasses a spectrum of parenchymal lung pathologies affecting the gas exchanging part of the lung. While many of these disease entities are not fibrotic in nature, a number can lead to pulmonary fibrosis which may or may not progress over time. Idiopathic pulmonary fibrosis is the prototypical, progressive fibrotic interstitial lung disease, which can lead to worsening hypoxemic respiratory failure and mortality within a number of years from the time of diagnosis. The importance of an accurate and timely diagnosis of interstitial lung diseases, which is needed to inform prognosis and guide clinical management, cannot be overemphasized. Developing a consensus diagnosis requires the incorporation of a variety of factors by a multidisciplinary team, which then may or may not determine a need for tissue sampling. Clinical management can be challenging given the heterogeneity of disease behavior and the paucity of controlled trials to guide decision making. This review addresses current paradigms and recent updates in the diagnosis and pharmacologic management of these fibrotic interstitial lung diseases. Full article
(This article belongs to the Special Issue Current Advances in Interstitial Lung Diseases)
22 pages, 735 KiB  
Review
Trials and Treatments: An Update on Pharmacotherapy for Idiopathic Pulmonary Fibrosis
by Lorraine Thong, Enda James McElduff and Michael Thomas Henry
Life 2023, 13(2), 486; https://doi.org/10.3390/life13020486 - 10 Feb 2023
Cited by 8 | Viewed by 5330
Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive fibrosing interstitial lung disease that occurs predominantly in the older population. There is increasing incidence and prevalence in IPF globally. The emergence of anti-fibrotic therapies in the last decade have improved patient survival though [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive fibrosing interstitial lung disease that occurs predominantly in the older population. There is increasing incidence and prevalence in IPF globally. The emergence of anti-fibrotic therapies in the last decade have improved patient survival though a cure is yet to be developed. In this review article, we aim to summarize the existing and novel pharmacotherapies for the treatment of IPF (excluding treatments for acute exacerbations), focusing on the current knowledge on the pathophysiology of the disease, mechanism of action of the drugs, and clinical trials. Full article
(This article belongs to the Special Issue Current Advances in Interstitial Lung Diseases)
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Other

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12 pages, 606 KiB  
Study Protocol
The Impact of Chronic Comorbidities on Outcomes in Acute Exacerbations of Idiopathic Pulmonary Fibrosis
by Saqib H. Baig and Erika J. Yoo
Life 2024, 14(1), 156; https://doi.org/10.3390/life14010156 - 21 Jan 2024
Viewed by 973
Abstract
Introduction: Idiopathic pulmonary fibrosis is a chronic progressive lung disease of unknown cause with a high associated mortality. We aimed to compare the impact of chronic medical conditions on hospital outcomes of patients with acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF). Methods: This [...] Read more.
Introduction: Idiopathic pulmonary fibrosis is a chronic progressive lung disease of unknown cause with a high associated mortality. We aimed to compare the impact of chronic medical conditions on hospital outcomes of patients with acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF). Methods: This was a retrospective cohort study using the NIS database from 2016 to 2018. We included patients aged 60 and older hospitalized in academic medical centers with the diagnoses of IPF and acute respiratory failure. We examined factors associated with hospital mortality and length of stay (LOS) using survey-weighted multivariate logistic and negative binomial regression. Results: Out of 4975 patients with AE-IPF, 665 (13.4%) did not survive hospitalization. There was no difference in the mean age between survivors and non-survivors. Patients were more likely to be male, predominantly white, and have Medicare coverage. Most non-survivors were from households with higher median income. Hospital LOS was longer among non-survivors than survivors (9.4 days vs. 9.8 days; p < 0.001). After multivariate-logistic regression, diabetes was found to be protective (aOR 0.62, 95% CI 0.50–0.77; p < 0.0001) while chronic kidney disease (CKD) conferred a significantly higher risk of death after AE-IPF (aOR 6.85, 95% CI 1.90–24.7; p = 0.00). Our multivariate adjusted negative binomial regression model for LOS identified obesity (IRR 0.85, 95% CI 0.76–0.94; p ≤ 0.00) and hypothyroidism (IRR 0.90, 95% CI 0.83–0.98; p = 0.02) to be associated with shorter hospital LOS. Conclusions: Our results suggest that CKD is a significant contributor to hospital mortality in AE-IPF, and diabetes mellitus may be protective. Obesity and hypothyroidism are linked with shorter hospital LOS among patients hospitalized with AE-IPF in US academic medical centers. Full article
(This article belongs to the Special Issue Current Advances in Interstitial Lung Diseases)
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