ijms-logo

Journal Browser

Journal Browser

Novel Immunomodulatory Approaches for Lung Fibrosis

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Immunology".

Deadline for manuscript submissions: closed (15 March 2024) | Viewed by 3104

Special Issue Editors


E-Mail Website
Guest Editor
Laboratory of Molecular Surfaces and Nanotechnologies (LAMSUN), Università di Catania, 295131 Catania, Italy
Interests: molecular biology; cell biology; cell adhesion fibroblasts; lung fibrosis; immunology and immunopharmacology

E-Mail Website
Guest Editor
Dipartimento di Medicina Clinica e Chirurgia, Università Degli Studi di Napoli Federico II, 80131 Napoli, Italy
Interests: interstitial lung diseases; idiopathic pulmonary fibrosis; immunity; cell biology; anti-fibrotic therapies

E-Mail Website
Guest Editor
Institute of Laboratory Medicine, German Center for Lung Research (DZL), Universities of Giessen and Marburg Lung Center (UGMLC), Philipps University Marburg, 235043 Marburg, Germany
Interests: IPF; MDSC; pharmacotherapy; immunomodulation; inflammation; fibrosis

Special Issue Information

Dear Colleagues,

Idiopathic pulmonary fibrosis (IPF) and the emerging clinical phenotype of rapidly progressive fibrosing interstitial lung diseases (f-ILDs) represent a treatment challenge because of their inexorable evolution despite the availability of anti-fibrotic drugs. The growing knowledge of the mechanisms involved in lung fibrogenesis has re-open the issue on the immune system involvement even though immunosuppressive therapies have been shown to be ineffective, if not harmful, in IPF, and similarly in f-ILDs. In the other hand, data in human and animal models have actually demonstrated that the dysregulation of both innate and adaptive immune responses is partly responsible for initiation and maintenance of the fibrotic process. Therefore, deciphering the role of immune players may allow the development of targeted and innovative immunomodulatory strategies to be explored in clinical practice, hopefully offering a wider range of therapeutic opportunities to an increasing number of fragile patients.

The aim of this Special Issue is to report the current knowledge of the role that different compartments of the immune system exert on fibrogenesis and illustrate novel immunomodulatory approaches to antifibrotic therapy. Therefore, we welcome authors to submit Original Research, Review, Mini-Review, Case Report, and Perspective articles focused on this inspiring topic.

Prof. Dr. Enrico Conte
Prof. Dr. Marialuisa Bocchino
Dr. Saeed Kolahian
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. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. There is an Article Processing Charge (APC) for publication in this open access journal. For details about the APC please see here. 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 (ILDs)
  • idiopathic pulmonary fibrosis (IPF)
  • immune dysregulation
  • therapies

Published Papers (2 papers)

Order results
Result details
Select all
Export citation of selected articles as:

Research

Jump to: Review

8 pages, 1138 KiB  
Communication
A Proof-of-Concept Analysis of Plasma-Derived Exosomal microRNAs in Interstitial Pulmonary Fibrosis Secondary to Antisynthetase Syndrome
by Sara Bozzini, Giovanni Zanframundo, Cecilia Bagnera, Eleonora Bozza, Sara Lettieri, Valentina Vertui, Veronica Codullo, Francesca Cuzzocrea, Belén Atienza-Mateo, Sara Remuzgo Martinez, Carlomaurizio Montecucco, Miguel A. González-Gay, Lorenzo Cavagna and Federica Meloni
Int. J. Mol. Sci. 2022, 23(23), 14579; https://doi.org/10.3390/ijms232314579 - 23 Nov 2022
Cited by 1 | Viewed by 1540
Abstract
Antisynthetase syndrome (ASSD) is an autoimmune disease characterized by the positivity of autoantibodies against different aminoacyl transfer RNA (tRNA) synthetases. Morbidity and mortality of this disease are highly affected by interstitial lung disease (ILD) which is present in about 80% of patients. In [...] Read more.
Antisynthetase syndrome (ASSD) is an autoimmune disease characterized by the positivity of autoantibodies against different aminoacyl transfer RNA (tRNA) synthetases. Morbidity and mortality of this disease are highly affected by interstitial lung disease (ILD) which is present in about 80% of patients. In this study, we investigated possible differences in 84 immune-related circulating miRNAs between ASSD patients with and without ILD; we enrolled 15 ASSD patients, 11 with ILD (ILD+) and 4 without ILD (ILD-), and 5 patients with idiopathic pulmonary fibrosis (IPF) as an additional control group. All patients were at disease onset and not on therapy at the time of inclusion. Differentially expressed miRNAs were identified in plasma-derived exosomes, using an miRNA PCR array (MIHS-111ZG, Qiagen, Hilden, Germany); miR-30a-5p and miR-29c-3p were upregulated in ASSD-ILD patients compared to patients without lung involvement (adjusted p-value < 0.05). IPF patients showed higher miR-29c-3p expression levels with respect to both ASSD and ASSD-ILD (p = 0.0005), whereas levels of miR-30a-5p were not different. miR-29c-3p and miR-30a-5p are overexpressed in ASSD-ILD+ patients compared with ILD−. These miRNAs are involved in the regulation of inflammation and fibrosis through their action on NF-κB and TGF-β1. Although the mechanistic role of these miRNAs in ASSD-ILD development has to be elucidated, we suggest that their exosome levels could be useful in identifying patients at risk of ILD. Full article
(This article belongs to the Special Issue Novel Immunomodulatory Approaches for Lung Fibrosis)
Show Figures

Figure 1

Review

Jump to: Research

14 pages, 326 KiB  
Review
Molecular Mechanism in the Development of Pulmonary Fibrosis in Patients with Sarcoidosis
by Elisabetta Cocconcelli, Nicol Bernardinello, Gioele Castelli, Simone Petrarulo, Serena Bellani, Marina Saetta, Paolo Spagnolo and Elisabetta Balestro
Int. J. Mol. Sci. 2023, 24(13), 10767; https://doi.org/10.3390/ijms241310767 - 28 Jun 2023
Cited by 2 | Viewed by 948
Abstract
Sarcoidosis is a multisystemic disease of unknown etiology characterized by the formation of granulomas in various organs, especially lung and mediastinal hilar lymph nodes. The clinical course and manifestations are unpredictable: spontaneous remission can occur in approximately two thirds of patients; up to [...] Read more.
Sarcoidosis is a multisystemic disease of unknown etiology characterized by the formation of granulomas in various organs, especially lung and mediastinal hilar lymph nodes. The clinical course and manifestations are unpredictable: spontaneous remission can occur in approximately two thirds of patients; up to 20% of patients have chronic course of the lung disease (called advanced pulmonary sarcoidosis, APS) resulting in progressive loss of lung function, sometimes life-threatening that can lead to respiratory failure and death. The immunopathology mechanism leading from granuloma formation to the fibrosis in APS still remains elusive. Recent studies have provided new insights into the genetic factors and immune components involved in the clinical manifestation of the disease. In this review we aim to summarize the clinical-prognostic characteristics and molecular pathways which are believed to be associated with the development of APS. Full article
(This article belongs to the Special Issue Novel Immunomodulatory Approaches for Lung Fibrosis)
Back to TopTop