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Advances in Pulmonary Hypertension and Interstitial Lung Diseases: Mechanisms and Treatments

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: closed (31 August 2025) | Viewed by 562

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
Special Issues, Collections and Topics in MDPI journals

E-Mail Website
Guest Editor
1st Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, 01-138 Warsaw, Poland
Interests: sarcoidosis; hypersensitivity pneumonitis; interstitial lung diseases; COVID-19 lung disease; pulmonary hypertension; mycobacterial lung diseases; pericardial diseases
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Pulmonary hypertension (PH) is a common complication of interstitial lung diseases (ILDs). The pathophysiology of PH associated with ILDs (PH-ILDs) is complex and includes pulmonary endothelium disfunction and pre-capillary vessel remodeling similar to that seen in pulmonary arteria hypertension (PAH). In the majority of ILDs, the patient’s PH remains mild; however, it can escalate severely in a small proportion of the patients. Worsening PH significantly degrades patients’ symptoms, quality of life, and survival.  Unfortunately, treatment options for PH-ILDs are limited, with only one medication targeting pulmonary vasculature approved for use in this group of patients—inhaled Treprostinil. A better understanding of many issues regarding PH-ILDs is necessary to improve patient management and outcomes. Some of them include alterations in pathways on molecular and cellular level, the identification of patients at risk of severe PH, the role of PAH-targeted medication and the real risk of hypoxemia with their use, or the role of antifibrotic treatment. Therefore, we invite researchers focusing on both basic and clinical sciences to share their thoughts and results from conducted studies. We look forward to receiving your contribution.

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

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Keywords

  • pulmonary hypertension
  • interstitial lung disease
  • lung fibrosis
  • pulmonary hemodynamics
  • pulmonary function testing
  • pathogenesis
  • prognosis
  • survival
  • treatment

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Published Papers (1 paper)

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Research

29 pages, 6176 KB  
Article
Initial Body Weight as an Important Factor for Improving the Reliability and Translational Relevance of the Preclinical Monocrotaline-Induced Rat Pulmonary Hypertension Model
by Patryk Remiszewski, Piotr Ryszkiewicz, Marta Baranowska-Kuczko, Anna Pędzińska-Betiuk, Krzysztof Mińczuk, Monika Kloza, Jolanta Weresa, Tomasz Hutsch and Barbara Malinowska
Int. J. Mol. Sci. 2025, 26(18), 8916; https://doi.org/10.3390/ijms26188916 - 12 Sep 2025
Viewed by 331
Abstract
Animal preclinical experiments in pulmonary hypertension (PH) need to be conducted with detailed methodological rigor to improve their translational relevance. One of its crucial yet insufficiently studied aspects is animal body weight (BW). Thus, our study aimed to examine the influence of initial [...] Read more.
Animal preclinical experiments in pulmonary hypertension (PH) need to be conducted with detailed methodological rigor to improve their translational relevance. One of its crucial yet insufficiently studied aspects is animal body weight (BW). Thus, our study aimed to examine the influence of initial BW on the severity of PH development induced by monocrotaline (MCT) and the effectiveness of the reference combined therapy (ambrisentan and tadalafil given for 21 days). Male rats were divided into three weight Sets: Set I (200–219 g); Set II (220–239 g); and Set III (240–259 g), after which, MCT-PH was induced. The measurements taken included in vivo echocardiographic evaluations, ex vivo functional experiments (on isolated right ventricle papillary muscles and pulmonary arteries), and histological and morphometric assessments. In all three Sets of animals, we noticed evidence of PH development. More pronounced changes confirming the severity of PH were observed in Set II compared to Sets I and III. The effectiveness of the reference therapy was also most evident in Set II, where the reversal of PH-related aggravations was best documented. We demonstrated that both the severity of MCT-induced PH in rats and the effectiveness of the reference combined therapy strongly depend on the animals’ initial BW. Full article
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