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Latest Updates about Interstitial Lung Disease

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".

Deadline for manuscript submissions: 20 January 2025 | Viewed by 828

Special Issue Editors


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Guest Editor
1st Department of Lung Diseases, National Research Institute of Tuberculosis and Lung Diseases, Płocka 26, 01-138 Warsaw, Poland
Interests: interstitial lung diseases; IPF; hypersensitivity pneumonitis; patients advocacy; patients related outcomes

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Guest Editor
First Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, 01-138 Warsaw, Poland
Interests: cardiac sarcoidosis; venous thromboembolism management and treatment; interstitial lung diseases, diagnosis and treatment; COVID-19 lung disease; pericardial diseases
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Special Issue Information

Dear Colleagues,

Interstitial lung diseases (ILDs) comprise more than 200 entities with different clinical, radiological, and pathological presentations, but they share one common feature—involvement of the lung interstitium. The prevalence of particular ILDs is low; they may be described as rare diseases according to the WHO definition. Nevertheless, morbidity and mortality of all ILDs are high, with hundreds of thousands of patients suffering and dying from them worldwide.

Some diseases, such as hypersensitivity pneumonitis or smoking-related interstitial fibrosis, have well-established etiological factors, but in most cases the etiology is unknown. Nevertheless, a genetic predisposition and aberrant molecular signaling are considered causes of many ILDs. On the other hand, some genetic mutations or polymorphisms may indicate more rapid disease progression or a worse response to treatment.

The most prevalent diagnoses are idiopathic pulmonary fibrosis, sarcoidosis, hypersensitivity pneumonitis, and connective-tissue disease-related ILD. The diagnostic pathway may be complicated with the need for specialized reference center involvement.

The treatment options are not very well established due to the difficulties in performing randomized clinical trials related to the small number of participants eligible.

Some of the ILDs are self-limiting or resolving after avoidance of the inciting agent. However, in around 30% of ILD patients a progressive fibrotic phenotype may develop, leading to the severe deterioration of these patients’ condition, respiratory failure, and death.

With regard to the above-mentioned problems, several new guidelines were developed in the last few years (i.e., guidelines for the diagnosis and treatment of idiopathic pulmonary fibrosis, recently updated with an additional part related to the progressive pulmonary fibrosis of other origin, and guidelines for the diagnosis and treatment of sarcoidosis and hypersensitivity pneumonitis).

Two antifibrotic drugs slowing the progression of fibrosis were developed in the last decade, but we as respiratory specialists still struggle with the problem of decreased survival and a lack of a cure for our patients.

To face the problems you meet in your everyday clinical practice and to help disseminate your research among a wide scientific audience, we invite you to share your original research articles, imaging studies, and review articles in the Special Issue of the International Journal of Molecular Sciences entitled “Latest Updates about Interstitial Lung Disease”.

This Special Issue will especially concern:

  1. Molecular and genetic disorders related to the pathogenesis of ILDs;
  2. New perspectives in the treatment of progressive pulmonary fibrosis (including personalized medicine);
  3. Environmental exposures as the risk factors of ILD development;
  4. Lung transplantation in patients with ILDs;
  5. Links between ILDs and pulmonary neoplasms;
  6. ILDs as a risk factor for thromboembolic disease;
  7. COVID-19 as a possible trigger of ILDs.

We hope that the published articles will contribute to the scientific discussion concerning these extremely interesting and still underexplored group of diseases.

Dr. Katarzyna B. Lewandowska
Prof. Dr. Monika Szturmowicz
Guest Editors

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Published Papers (1 paper)

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Research

32 pages, 6199 KiB  
Article
Beneficial Impact of Inhaled 25(OH)-Vitamin D3 and 1,25(OH)2-Vitamin D3 on Pulmonary Response in the Murine Model of Hypersensitivity Pneumonitis
by Marta Kinga Lemieszek, Michał Chojnacki, Iwona Paśnik, Wiktoria Gawryś, Alicja Wilczyńska, Ilona Leśniowska and Jakub Anisiewicz
Int. J. Mol. Sci. 2024, 25(19), 10289; https://doi.org/10.3390/ijms251910289 - 24 Sep 2024
Viewed by 428
Abstract
Despite numerous scientific reports on the negative impact of vitamin D3 deficiency on many respiratory diseases, little is known about the influence of this phenomenon on the development and progression of hypersensitivity pneumonitis (HP). The presented study is an attempt to shed light [...] Read more.
Despite numerous scientific reports on the negative impact of vitamin D3 deficiency on many respiratory diseases, little is known about the influence of this phenomenon on the development and progression of hypersensitivity pneumonitis (HP). The presented study is an attempt to shed light on this occurrence. The research was performed on mouse strain C57BL/6J exposed to the antigen of Pantoea agglomerans (etiological factor of HP). To induce vitamin D3 deficiency, mice received a diet with a 10 times lower amount of cholecalciferol than the main control group. VD3-deficient mice inhaled 25(OH)-VD3 or 1,25(OH)2-VD3 used separately or with SE-PA. At the beginning of the experiment and after 14 and 28 days of inhalation, respiratory function was examined using whole-body plethysmography. Moreover, at indicated time points, mice were sacrificed and samples collected for histological examination, flow cytometry, and ELISA. The performed study revealed that inhalations with 25(OH)-VD3 and 1,25(OH)2-VD3 effectively eliminated most of the negative changes in the respiratory system caused by vitamin D3 deficiency by restoring the physiological concentration of 1,25(OH)2-VD3 in the body. VD3-deficient mice which inhaled P. agglomerans antigen and vitamin D3 metabolites also demonstrated the ability of the tested compounds to eliminate, or at least weaken, the negative effects of the HP causative factor and desired effect, including improvement of respiratory functions and attenuation of inflammation and signs of fibrosis. The obtained results suggested that the beneficial influence of inhaled vitamin D3 metabolites on HP development was associated with the restoration of the physiological concentration of 1,25(OH)2-VD3 in the pulmonary compartments in VD3-deficient mice. Full article
(This article belongs to the Special Issue Latest Updates about Interstitial Lung Disease)
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