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Medicina
Special Issues
Diagnosis and Treatment of Interstitial Lung Disease
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Diagnosis and Treatment of Interstitial Lung Disease

A special issue of Medicina (ISSN 1648-9144). This special issue belongs to the section "Pulmonology".

Deadline for manuscript submissions: closed (31 July 2025) | Viewed by 4932

Special Issue Editor

Prof. Dr. Roberto G. Carbone

E-Mail Website
Guest Editor
Department of Internal Medicine, University of Genoa, Viale Benedetto XV, 6, 16132 Genoa, Italy
Interests: respiratory diseases; respiratory pathophysiology; COPD; interstitial lung diseases; pulmonary hypertension; lung transplantation
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Interstitial lung disease (ILD) comprises a large group of over 200 parenchymal pulmonary diseases characterized by progressive lung fibrosis. The most common ILDs are non-specific interstitial pneumonia, usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF), hypersensitivity pneumonia, and pulmonary sarcoidosis, while rare forms include desquamative interstitial pneumonia, pulmonary Langerhans cell histiocytosis, and lymphangio-leiomyomatosis. Each ILD subtype has distinctive radiographic and pathologic features, with interventional endoscopic procedures such as bronchoscopy, allowing for trans-bronchial biopsies, broncho-alveolar lavage fluid recovery, and cryo-biopsy facilitating accurate diagnoses. Pulmonary arterial hypertension (Group 1) and pulmonary hypertension (Group 3) are frequently detected in patients with ILDs. Treatment includes high steroid doses, antibiotics such as azithromycin, high-flow O2 support, non-invasive ventilation, and, more recently, anti-fibrotic drugs, like pirfenidone and nintedanib. Meanwhile, lung transplantation is the elective treatment for end-stage ILDs.

Current problems include the following: (1) difficulty to control severe exacerbations; (2) analysis of disease progression and outcomes; (3) efficacy of vaso-dilator drugs in pulmonary hypertension; and (4) identification of an accurate composite allocation score.

This Special Issue aims to focus on recent aspects of basic and clinical ILD research.

Suggested topics include the following:

  • UIP/IPF genome analysis;
  • IPF epigenetics;
  • Artificial intelligence in high-resolution computed tomography (HRCT) for ILD diagnosis;
  • Endobronchial ultrasound and cryo-biopsy in ILD;
  • Connective tissue disease-associated ILD;
  • Post-COVID19 ILD;
  • Interstitial lung abnormalities (ILAs).

Prof. Dr. Roberto G. Carbone
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 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. Medicina 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 2200 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
  • echocardiography
  • right heart catheterization
  • bronchoscopy
  • HRCT

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Published Papers (3 papers)

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15 pages, 1109 KB  
Article
Assessing Fibrosis Progression and Endothelial Dysfunction in SSc-ILD and COPD: An Integrated Biomarker and CT Densitometry Approach
by Lyazat Ibrayeva, Irina Bacheva, Assel Alina and Olga Klassen
Medicina 2025, 61(9), 1572; https://doi.org/10.3390/medicina61091572 - 31 Aug 2025
Viewed by 351
Abstract
Background and Objectives: Chronic lung diseases act as multi-organ conditions in which systemic inflammation, vascular dysfunction, and fibrosis intersect. The pulmo-renal continuum—functional crosstalk between lungs and kidneys—remains poorly characterized. We compared year-long changes in endothelin-1 (ET-1), galectin-3 (Gal-3), renal indices (eGFR, ACR), and [...] Read more.
Background and Objectives: Chronic lung diseases act as multi-organ conditions in which systemic inflammation, vascular dysfunction, and fibrosis intersect. The pulmo-renal continuum—functional crosstalk between lungs and kidneys—remains poorly characterized. We compared year-long changes in endothelin-1 (ET-1), galectin-3 (Gal-3), renal indices (eGFR, ACR), and quantitative CT densitometry in COPD and systemic sclerosis-associated ILD (SSc-ILD). Materials and Methods: In this prospective observational study (January 2023–December 2024), 112 patients were consecutively enrolled (COPD, n = 58; SSc-ILD, n = 54). Assessments were performed at baseline and 12 months. ET-1 (ELISA) and Gal-3 (chemiluminescence) were measured in serum; eGFR was calculated by the creatinine-based CKD-EPI (2021) equation; ACR was photometric. High-resolution chest CT provided lung volume and parenchymal density (Hounsfield units) at six predefined axial levels per lung. Non-parametric statistics were applied: Wilcoxon signed-rank (within-group), Mann–Whitney U (between-group), and Spearman rank correlations for associations; results are reported with p-values (and 95% CIs). Results: Baseline eGFR was normal (COPD 90.37; SSc-ILD 92.4 mL/min/1.73 m2). eGFR declined by 6.76% in COPD (p = 0.001) and 3.16% in SSc-ILD (p = 0.029). ET-1 increased in both cohorts but more in COPD (+83.78%, p = 0.0002) than in SSc-ILD (+23.83%, p = 0.0001). Gal-3 rose significantly only in SSc-ILD (+10.2%, p = 0.043). FVC decreased in COPD (−4.01%, p = 0.01) and was unchanged in SSc-ILD. Total lung volume declined in SSc-ILD (−6.08%, p = 0.02) but not in COPD. CT density shifts were small: several slices in COPD and one slice (L6) in SSc-ILD reached statistical but not biological relevance. Conclusions: COPD exhibited larger vascular and renal biomarker shifts (ET-1 up, eGFR down, ACR up), suggesting systemic inflammation and early renal involvement. In SSc-ILD, biomarker and CT changes predominantly reflected pulmonary fibrosis progression with limited renal impact. Integrating biomarkers with quantitative CT may help delineate organ-specific trajectories along the pulmo-renal continuum; longer, larger studies are warranted. Limitations: This was a single-center cohort with a modest sample (58 COPD and 54 SSc-ILD) and a 12-month, two-time-point follow-up, which may not capture long-term trajectories and may limit it generalizability; larger multicenter studies with an extended follow-up are warranted. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Interstitial Lung Disease)
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7 pages, 1422 KB  
Article
Exploring the Potential of a P2X3 Receptor Antagonist: Gefapixant in the Management of Persistent Cough Associated with Interstitial Lung Disease
by Tomoyuki Takahashi, Atsushi Saito, Takafumi Yorozuya, Hirotaka Nishikiori, Koji Kuronuma and Hirofumi Chiba
Medicina 2025, 61(5), 892; https://doi.org/10.3390/medicina61050892 - 14 May 2025
Viewed by 1245
Abstract
Background: Interstitial lung disease (ILD) is characterized by pulmonary inflammation and fibrosis associated with persistent and refractory cough that significantly hinders quality of life. Conventional treatments for ILD-associated cough have shown limited efficacy, necessitating alternative therapeutic approaches. Gefapixant, a P2X3 receptor antagonist, can [...] Read more.
Background: Interstitial lung disease (ILD) is characterized by pulmonary inflammation and fibrosis associated with persistent and refractory cough that significantly hinders quality of life. Conventional treatments for ILD-associated cough have shown limited efficacy, necessitating alternative therapeutic approaches. Gefapixant, a P2X3 receptor antagonist, can potentially alleviate chronic cough by inhibiting the ATP-mediated activation of sensory C-fibers, but its efficacy in ILD-associated cough remains unclear. This study observed the effects of gefapixant on ILD-associated refractory chronic cough. Methods: This prospective study enrolled patients with ILD-associated refractory chronic cough who received gefapixant at Sapporo Medical University Hospital between July 2022 and November 2023. Cough frequency, Leicester Cough Questionnaire (LCQ) score, cough severity visual analog scale (Cough VAS), and taste VAS were evaluated at baseline and at 2, 4, and 8 weeks after gefapixant administration. Results: Six patients completed the study. Their ILD subtypes included idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), and connective tissue disease-associated ILDs (CTD-ILDs). After 8 weeks, the cough frequency decreased from 88.5 to 44.3 episodes per 30 min, LCQ scores increased from 8.3 to 13.6, and cough VAS scores decreased from 75.8 to 40.2. However, statistical significance was not reached due to high interindividual variability, with gefapixant being effective in some and ineffective in others. The most common adverse event was taste disorder, leading to discontinuation in one patient, but symptoms tended to lessen over the course of treatment. Conclusions: Gefapixant appears to be effective in reducing refractory cough related to ILD, although these results were not statistically significant because its effectivity widely varied across individuals. Further investigation is needed to identify patient subgroups with the greatest potential for treatment responsiveness. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Interstitial Lung Disease)
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9 pages, 1883 KB  
Article
Comparison of the Effects of Nintedanib and Pirfenidone on Pulmonary Function Test Parameters and Radiological Findings in Patients with Idiopathic Pulmonary Fibrosis: A Real-Life Study
by Olcay Aycicek, Serra Keskin, Muhammed Haciosmanoglu, Funda Oztuna, Yilmaz Bulbul and Tevfik Ozlu
Medicina 2025, 61(2), 283; https://doi.org/10.3390/medicina61020283 - 6 Feb 2025
Cited by 1 | Viewed by 2925
Abstract
Background and Objectives: The aim of our study is to compare the effects of pirfenidone and nintedanib on lung function and radiologic findings in Idiopathic Pulmonary Fibrosis and to identify which drug is more appropriate for which patient group. Materials and Methods: The [...] Read more.
Background and Objectives: The aim of our study is to compare the effects of pirfenidone and nintedanib on lung function and radiologic findings in Idiopathic Pulmonary Fibrosis and to identify which drug is more appropriate for which patient group. Materials and Methods: The data of patients who were treated in our department for at least one year between 1 January 2010 and 31 December 2022 and who were started on pirfenidone or nintedanib treatment with the diagnosis of Idiopathic Pulmonary Fibrosis were retrospectively reviewed. The patients were divided into two groups—the nintedanib and pirfenidone groups—and both groups were compared in terms of progression in lung function tests (changes in FEV1, FVC, 6 MWT and DLCO values at the 3rd, 6th, 9th and 12th months compared to baseline values) and radiological findings (the presence of progression in findings such as ground-glass opacity, reticulation, honeycomb and traction bronchiectasis) within 1 year after diagnosis. Results: The study included 109 patients. The number of patients treated with pirfenidone (IPF patients) was 82 (75.2%) and the number of patients treated with nintedanib was 27 (24.8%). When the PFT values at 3, 6, 9 and 12 months were compared with the baseline values in both groups, there was no statistically significant difference in any parameter between the two groups. No significant difference was found in terms of radiological progression at the end of 1 year in both groups. Conclusions: The results of our study show that pirfenidone and nintedanib are equivalent in their effectiveness in preventing disease progression in patients with IPF. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Interstitial Lung Disease)
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