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Advances in Pulmonary Hypertension and Idiopathic Pulmonary Fibrosis
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Advances in Pulmonary Hypertension and Idiopathic Pulmonary Fibrosis

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

Deadline for manuscript submissions: 20 November 2025 | Viewed by 1057

Special Issue Editor

Prof. Dr. Roberto Carbone

E-Mail Website
Guest Editor
Department of Internal Medicine, University of Genoa, Genoa, Italy
Interests: interstitial lung disease; pulmonary hypertension; lung transplant; pulmonary vascular disorders
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Pulmonary hypertension leads to cardiac dysfunction and pulmonary remodeling. The main consequences of pulmonary hypertension are, at the cardiac level, left heart failure and mitral valve disease and, at the pulmonary level, chronic hypoxemia and structural lung alterations.

Pulmonary hypertension is divided in five groups according to the European Society of Cardiology (ESC)/American Heart Association (AHA) guidelines. Pulmonary hypertension associated with idiopathic pulmonary fibrosis, the most common form, and interstitial lung disease, the form with the worse prognosis, belong to Group 3 of the ESC/AHA classification.

This Special Issue will describe the epidemiology, pathophysiology, immunology, imaging, diagnosis, outcome and treatment of pulmonary hypertension associated with idiopathic pulmonary fibrosis.

Prof. Dr. Roberto 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. 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

  • pulmonary hypertension
  • interstitial lung disease
  • idiopathic pulmonary fibrosis
  • heart failure
  • mitral valve disease

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Published Papers (2 papers)

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Research

18 pages, 1358 KB  
Article
Pulmonary Hypertension Drives Prognosis in Idiopathic Pulmonary Fibrosis: Insights from the European IPF Registry
by Andreas Guenther, Silke Tello, Marc Carre Schoppe, Joern Pons-Kuehnemann, Werner Seeger, Johannes Stiben, Khodr Tello, Maria Molina Molina, Carlo Vancheri, Bruno Crestani and Ekaterina Krauss
J. Clin. Med. 2025, 14(20), 7352; https://doi.org/10.3390/jcm14207352 - 17 Oct 2025
Viewed by 281
Abstract
Background/Objectives: In patients with idiopathic pulmonary fibrosis (IPF), a progressive disease characterized by lung tissue scarring, the impact of comorbidities is only partially understood. In particular, the prognostic implications of pulmonary hypertension (PH) are yet to be fully disclosed. Methods: To [...] Read more.
Background/Objectives: In patients with idiopathic pulmonary fibrosis (IPF), a progressive disease characterized by lung tissue scarring, the impact of comorbidities is only partially understood. In particular, the prognostic implications of pulmonary hypertension (PH) are yet to be fully disclosed. Methods: To identify distinct IPF phenotypes on the basis of comorbidities and functional data, we performed cluster mixed data retrospective analysis, as well as recursive partitioning analysis on a dataset of 324 patients from the European IPF Registry (eurIPFreg); all patients were classified as IPF on the basis of established guidelines. Diagnosis of PH was based on echocardiographic and right heart catheter criteria as indicated in the 2022 ESC/ERS guidelines. Results: Two distinct clinical clusters with significant survival differences were identified (p < 0.001). Cluster 1, with fewer comorbidities, had a median survival of 4.41 years, whereas Cluster 2, with higher rates of arterial hypertension, diabetes mellitus, cardiovascular disease, PH, and dyslipidemia, showed a shorter median survival of 2.85 years. Multivariate Cox regression analysis confirmed PH as a significant predictor of reduced survival (HR 2.03). Recursive partitioning (RP) revealed that FVC was the strongest prognostic indicator: FVC below 50% predicted poor survival, and among patients with a FVC above 50%, the presence of PH indicated a significantly worse outcome. Conclusions: In this real-world IPF cohort, comorbidity cluster and RP analysis identified PH as the most relevant comorbidity. The findings suggest that PH may be more prevalent and impactful in IPF than previously recognized, with implications for clinical management. Full article
(This article belongs to the Special Issue Advances in Pulmonary Hypertension and Idiopathic Pulmonary Fibrosis)
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12 pages, 765 KB  
Article
Development and Validation of a Scoring System for Prediction of Tolerance to Inhaled Treprostinil in Patients with PAH or PH-ILD
by Alan Lanurias Diaz, Ashwin Kumar, Obada Kholoki, David M. O’Sullivan, Kristen Swanson, Brett Carollo, Joseph Bahgat, Harrison W. Farber and Raj Parikh
J. Clin. Med. 2025, 14(18), 6395; https://doi.org/10.3390/jcm14186395 - 10 Sep 2025
Viewed by 583
Abstract
Background: Treprostinil has demonstrated effectiveness in treating Pulmonary Arterial Hypertension (PAH) and Pulmonary Hypertension associated with Interstitial Lung Disease (PH-ILD). However, tolerability remains a clinical challenge. Identifying factors influencing tolerability is important, given the adverse outcomes of PAH and PH-ILD and the [...] Read more.
Background: Treprostinil has demonstrated effectiveness in treating Pulmonary Arterial Hypertension (PAH) and Pulmonary Hypertension associated with Interstitial Lung Disease (PH-ILD). However, tolerability remains a clinical challenge. Identifying factors influencing tolerability is important, given the adverse outcomes of PAH and PH-ILD and the potential of treprostinil to slow disease progression. Objective: This study was undertaken to identify tolerance factors and develop a predictive scoring system. Methods: A retrospective analysis of 65 patients (37 PAH, 28 PH-ILD) was conducted using patient history, pulmonary function tests (PFTs), transthoracic echocardiograms (TTEs), and right heart catheterizations (RHCs). Of these, 67.7% (n = 44) tolerated treprostinil, while 32.3% (n = 21) were intolerant. Results: Patients who tolerated treprostinil had better pulmonary function, with a higher forced expiratory volume in one second/forced vital capacity (FEV1/FVC) ratio (82.27 ± 16.06 vs. 72.86 ± 17.76, p = 0.037) and superior right ventricular function, as indicated by higher tricuspid annular plane systolic excursion (TAPSE: 2.05 ± 0.37 vs. 1.64 ± 0.42, p < 0.001), higher cardiac index (CI: 2.51 ± 0.67 vs. 2.03 ± 0.53, p = 0.003), and improved functional status (p < 0.001). The Inhaled Treprostinil Intolerance Score (ITIS), incorporating TAPSE < 1.6, CI < 2, FEV1/FVC < 70%, and WHO functional class (FC) 3 or 4, demonstrated strong predictive accuracy (cutoff ≥ 2, AUC = 0.884 ± 0.048, p < 0.001). Predictive performance was stronger in PAH patients (AUC = 0.921 ± 0.053) than PH-ILD (AUC = 0.833 ± 0.093, p < 0.001). Conclusions: These findings demonstrate the importance of clinical parameters in predicting treprostinil tolerance. Further investigation is warranted to refine the scoring system, particularly for PH-ILD patients. Full article
(This article belongs to the Special Issue Advances in Pulmonary Hypertension and Idiopathic Pulmonary Fibrosis)
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