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Interstitial Lung Diseases: New Treatments and Future Directions
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Interstitial Lung Diseases: New Treatments and Future Directions

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Respiratory Medicine".

Deadline for manuscript submissions: 30 April 2026 | Viewed by 7852

Special Issue Editor

Dr. Justyna Fijołek

E-Mail Website
Guest Editor
The Third Department of Pneumonology and Oncology, National Tuberculosis and Lung Diseases Research Institute, Plocka St. 26, 01-138 Warsaw, Poland
Interests: interstitial lung disease; ANCA-associated vasculitis; orphan lung disease; interstitial pneumonia; rheumatic lung disease; chronic respiratory disorders

Special Issue Information

Dear Colleagues,

Interstitial lung diseases (ILDs) are a diverse group of disorders that lead to progressive lung fibrosis and significant morbidity. These diseases present a considerable challenge in clinical practice due to their heterogeneity and complex pathophysiology. This Special Issue, titled "Interstitial Lung Diseases: New Treatments and Future Directions", will focus on recent breakthroughs in the treatment of ILDs, highlighting novel therapeutic approaches and emerging treatment strategies aimed at improving patient outcomes.

Key topics of interest will include the following:

  • The latest advancements in pharmacologic treatments, such as antifibrotic agents, immunomodulators, and biologic therapies;
  • The role of precision medicine in ILDs, exploring individualized treatment approaches based on genetic, phenotypic, and environmental factors;
  • Investigations into novel drug mechanisms and combination therapies designed to halt or reverse disease progression;
  • The potential impact of emerging treatments, including regenerative medicine, stem cell therapy, and gene therapies, in the future management of ILDs;
  • Future challenges in the long-term management and development of patient-centered care strategies.

This Special Issue aims to provide an in-depth exploration of the evolving landscape of ILD treatment, offering insights into both established and emerging therapies. We invite contributions that focus on innovative treatment strategies, the scientific basis for these interventions, and their potential to transform the management of ILDs.

Dr. Justyna Fijołek
Guest Editor

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 250 words) can be sent to the Editorial Office for assessment.

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. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly 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 Disease (ILD)
  • pulmonary fibrosis
  • orphan lung disease
  • AAV-ILD
  • occupational ILD
  • rheumatic lung disease
  • treatment strategies
  • emerging therapies
  • precision medicine
  • future directions

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Further information on MDPI's Special Issue policies can be found here.

Published Papers (2 papers)

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Review

33 pages, 822 KB  
Review
Artificial Intelligence Enabled Lung Sound Auscultation in the Early Diagnosis and Subtyping of Interstitial Lung Disease
by Avneet Kaur, Swathi Priya Cherukuri, Megha Shashidhar Handral, Hanisha Reddy Kukunoor, Rikesh KC, Swathi Godugu, Jieun Lee, Gayathri Yerrapragada, Poonguzhali Elangovan, Mohammed Naveed Shariff, Thangeswaran Natarajan, Jayarajasekaran Janarthanan, Jayavinamika Jayapradhaban Kala, Sancia Mary Jerold Wilson, Samuel Richard, Shiva Sankari Karrupiah, Dipankar Mitra, Vivek N. Iyer, Scott A. Helgeson and Shivaram P. Arunachalam
J. Clin. Med. 2025, 14(23), 8500; https://doi.org/10.3390/jcm14238500 - 30 Nov 2025
Cited by 1 | Viewed by 1745
Abstract
Background: Interstitial lung disease (ILD) involves numerous chronic pulmonary conditions that damage the lung parenchyma and alveolar interstitium. ILD has overlapping clinical and radiological features with other commonly seen cardiac and respiratory conditions. If not identified and treated in a timely manner, it [...] Read more.
Background: Interstitial lung disease (ILD) involves numerous chronic pulmonary conditions that damage the lung parenchyma and alveolar interstitium. ILD has overlapping clinical and radiological features with other commonly seen cardiac and respiratory conditions. If not identified and treated in a timely manner, it may lead to irreversible fibrosis and a poor prognosis in the patient. The current diagnostic methods are either invasive or reliant on imaging or specialist interpretation, which can lead to diagnostic delay, increased radiation exposure, and healthcare costs. Lung crackles, often under-recognized as a non-specific feature of ILD, may serve as an important diagnostic clue in identifying not only the early stages of ILD but also its subtypes. This review explores the potential of analyzing the lung sounds in ILD through AI-based auscultation. Objective: To provide a comprehensive analysis of the pathophysiological stages of lung injury in ILD, the specific acoustic features, and the location associated with each ILD subtype and to evaluate the current state-of-the-art non-AI and AI methodologies that are used to diagnose ILD. This review aims to analyze the limitations associated with the current modalities and to envision AI-integrated auscultation as a powerful, cost-effective, non-invasive, radiation-free screening tool for early detection of ILD and its subtypes. Content Overview: The review begins with a detailed analysis of the lung sound pathophysiology, exploring the two-stage mechanism of alveolar epithelial injury and fibrosis formation. Existing hypotheses explaining the mechanism behind crackle production and the role of structural anatomy and surface tension in the generation of pathological lung sounds are examined. A tabulated summary of common ILD subtypes is provided, including their inciting events, pathogenesis, anatomical auscultation locations, and prognostic implications. Current diagnostic modalities for ILD, both non-AI and AI-based, are summarized along with their limitations, emphasizing the need for improved diagnostic tools. Discussion: Existing studies suggest that AI-based auscultation can match or exceed the current modalities in its sensitivity and specificity for detecting ILD-related crackles. Clinicians can identify the specific sound pattern and then correlate it with the ILD subtype and understand the prognosis in real time, thereby providing timely intervention to the patient. Additionally, AI-based auscultation can be used in resource-limited settings and can potentially reduce dependence on pulmonology expertise and radiation-based imaging for monitoring the condition. Conclusions: This literature review highlights the clinical potential of AI-based auscultation for early and accurate diagnoses of ILD. Understanding the associated pathological sounds, biomarkers, and genetic mutations linked to different subtypes opens avenues for future development of non-invasive diagnostic panels for ILD in clinical practice. Full article
(This article belongs to the Special Issue Interstitial Lung Diseases: New Treatments and Future Directions)
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31 pages, 2674 KB  
Review
Clinical Insights and Therapeutic Strategies for the Treatment of Interstitial Lung Disease in Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: Current Trends and Future Directions
by Justyna Fijolek and Anna Sniady
J. Clin. Med. 2025, 14(13), 4631; https://doi.org/10.3390/jcm14134631 - 30 Jun 2025
Cited by 2 | Viewed by 5027
Abstract
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and interstitial lung disease (ILD) represent a complex interplay between autoimmune and fibrotic processes that poses significant diagnostic and therapeutic challenges. The distinction between isolated ANCA-ILD and AAV-ILD remains a subject of ongoing debate, with some researchers [...] Read more.
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and interstitial lung disease (ILD) represent a complex interplay between autoimmune and fibrotic processes that poses significant diagnostic and therapeutic challenges. The distinction between isolated ANCA-ILD and AAV-ILD remains a subject of ongoing debate, with some researchers proposing that ANCA-ILD may be an early or restricted form of systemic vasculitis. Immunosuppressive therapy is the cornerstone of treatment for both diseases. However, there is increasing evidence that supports the potential role of antifibrotic agents in the management of progressive fibrosis. Management of these diseases requires a personalized approach that incorporates evaluation of biomarkers, imaging findings, and clinical risk factors to guide treatment decisions. Although current therapeutic strategies primarily target systemic inflammation, addressing the fibrotic components of these diseases is crucial for improving outcomes. Furthermore, emerging therapeutic options, such as B-cell depletion and antifibrotic therapies, offer promising outcomes. However, their roles in the treatment of AAV-ILD require further exploration. In this review, we discuss clinical insights and evolving therapeutic strategies for managing AAV and ANCA-positive ILD. In addition, we highlight the importance of early diagnosis and individualized treatment plans in improving the prognosis and quality of life of affected patients. Full article
(This article belongs to the Special Issue Interstitial Lung Diseases: New Treatments and Future Directions)
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