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Life
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Advances in Pulmonary Fibrosis
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Advances in Pulmonary Fibrosis

A special issue of Life (ISSN 2075-1729). This special issue belongs to the section "Medical Research".

Deadline for manuscript submissions: closed (2 December 2024) | Viewed by 2044

Special Issue Editors

Dr. Mary M. Salvatore

E-Mail Website
Guest Editor
Department of Radiology, NewYork-Presbyterian, Columbia University Irving Medical Center, New York, NY 10032, USA
Interests: targeted therapies; molecular diagnostics; tumor Immunity
Special Issues, Collections and Topics in MDPI journals
Dr. Maria Padilla

E-Mail Website
Guest Editor
Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA
Interests: idiopathic pulmonary fibrosis; sarcoidosis; collagen vascular associated pulmonary fibrosis; genetic associated pulmonary fibrosis; hermansky pudlak syndrome; bronchiolitis obliterans

Special Issue Information

Dear Colleagues,

It has been nearly 10 years since the approval of the first and only antifibrotic medications for the treatment of pulmonary fibrosis. The field has been active in investigating the clinical manifestations, imaging and pathological findings of interstitial lung diseases, particularly pulmonary fibrosis. Overall, novel molecular, genetic, genomic and proteomics advances have enhanced our understanding of the pathogenesis of disease and have provided biomarkers that make precision medicine a possibility for the management of interstitial lung diseases and therapeutic interventions. Diagnostic, prognostic and theragnostic biomarkers facilitate earlier and targeted diagnoses, predict the course of illness and allow therapy selection that specifically addresses the biology of the disease. This approach has made a significant difference in the oncology and asthma areas. We are excited to bring together cutting-edge clinicians and scientists to build upon the strides of the last decade. We are seeking manuscripts that aim to propel the field forward. We are looking for clinically applicable articles that address the screening of high-risk populations, establish diagnoses, stratify the risk of disease progression, guide therapeutic selection and management, and assess co-morbidities. These can include radiology tools that not only identify early disease but also the likelihood of progression. Novel AI algorithms hold promise in this regard and thus need to be validated. Screening techniques that will address the early detection of co-morbidities such as lung cancer are imperative. Clinical and serologic biomarkers that predict disease development and course of illness will be important contributions. Ultimately, we hope that this collection will address the current challenges and provide a road map for the next ten years that will lead to better outcomes for our patients.

Dr. Mary M. Salvatore
Dr. Maria Padilla
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. Life 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 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

  • lung fibrosis
  • biomarkers
  • artificial intelligence
  • lung cancer in pulmonary fibrosis

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Published Papers (2 papers)

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Research

21 pages, 2076 KiB  
Article
The Nutritional Phenotyping of Idiopathic Pulmonary Fibrosis Through Morphofunctional Assessment: A Bicentric Cross-Sectional Case–Control Study
by Alicia Sanmartín-Sánchez, Rocío Fernández-Jiménez, Eva Cabrera-César, Francisco Espíldora-Hernández, Isabel Vegas-Aguilar, María del Mar Amaya-Campos, Fiorella Ximena Palmas-Candia, Josefina Olivares-Alcolea, Víctor José Simón-Frapolli, Isabel Cornejo-Pareja, Ana Sánchez-García, Mora Murri, Patricia Guirado-Peláez, Álvaro Vidal-Suárez, Lourdes Garrido-Sánchez, Francisco J. Tinahones, Jose Luis Velasco-Garrido and Jose Manuel García-Almeida
Life 2025, 15(4), 516; https://doi.org/10.3390/life15040516 - 21 Mar 2025
Viewed by 429
Abstract
There is increasing evidence supporting the use of morphofunctional assessment (MFA) as a tool for clinical characterization and decision-making in malnourished patients. MFA enables the diagnosis of malnutrition, sarcopenia, obesity, and cachexia, leading to a novel phenotype-based classification of nutritional disorders. Bioelectrical impedance [...] Read more.
There is increasing evidence supporting the use of morphofunctional assessment (MFA) as a tool for clinical characterization and decision-making in malnourished patients. MFA enables the diagnosis of malnutrition, sarcopenia, obesity, and cachexia, leading to a novel phenotype-based classification of nutritional disorders. Bioelectrical impedance analysis (BIVA), nutritional ultrasound® (NU) and computed tomography (CT) are included, along with functional tests like the Timed Up and Go test (TUG). Myoesteatosis, detectable via CT, can occur independently from nutritional phenotypes and has been identified as a significant mortality predictor in idiophatic pulmonary fibrosis (IPF). Our aim is to analyze the prevalence and overlap of nutritional phenotypes in IPF and evaluate the prognostic value of myoesteatosis. Our bicenter cross-sectional study included 82 IPF patients (84.1% male and with a medium age of 71.1 ± 7.35 years). MFA was performed using BIVA, NU, CT at the T12 level (CT-T12), the handgrip strength (HGS) test, and the TUG. CT-T12 BC parameters were analyzed using FocusedON® software, while statistical analyses were conducted with JAMOVI version 2.3.22. All four major nutritional phenotypes were represented in our cohort, with significant overlap. A total of 80.5% met the GLIM criteria for malnutrition, 14.6% had cachexia, 17% were sarcopenic, and 28% were obese. Of the obese patients, 70% were also malnourished, while 100% of sarcopenic obese patients (5.9% of total) had malnutrition. A total of 55% of sarcopenic patients with available CT also had myosteatosis, suggesting muscle quality deterioration as a potential driver of functional impairment. The presence of myosteatosis > 15% in T12-CT was an independent predictor of 12-month mortality (HR = 3.13; 95% CI: 1.01–9.70; p = 0.049), with survival rates of 78.1% vs. 96.6% in patients with vs. without myosteatosis, respectively. To conclude, this study underscores the relevance of MFA in the nutritional characterization of patients with IPF, demonstrating its potential to identify specific phenotypes associated with malnutrition, functional impairment, and the presence of myoesteatosis, thereby providing a valuable tool for clinical decision-making. Full article
(This article belongs to the Special Issue Advances in Pulmonary Fibrosis)
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13 pages, 1028 KiB  
Article
Gender Differences Are a Leading Factor in 5-Year Survival of Patients with Idiopathic Pulmonary Fibrosis over Antifibrotic Therapy Reduction
by Pasquale Tondo, Giulia Scioscia, Cosimo C. De Pace, Fabiola Murgolo, Federica Maci, Giulia M. Stella, Dalila Pescatore, Maria Pia Foschino Barbaro and Donato Lacedonia
Life 2025, 15(1), 106; https://doi.org/10.3390/life15010106 - 16 Jan 2025
Viewed by 1092
Abstract
Background: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease with a median survival of 3–5 years. Antifibrotic therapies like pirfenidone and nintedanib slow progression, but the outcomes vary. Gender may influence disease presentation, progression, and response to treatment. This study evaluates [...] Read more.
Background: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease with a median survival of 3–5 years. Antifibrotic therapies like pirfenidone and nintedanib slow progression, but the outcomes vary. Gender may influence disease presentation, progression, and response to treatment. This study evaluates the impact of gender on the 5-year survival, pharmacological management, and clinical outcomes of patients with IPF. Methods: A retrospective cohort study of 254 IPF patients was conducted, with 164 (131 males:33 females) having complete data. Patients underwent spirometry, DLCO, and 6 min walk tests. Data on comorbidities, smoking, antifibrotic therapy type, dosage adjustments, and adverse events were collected. We used Kaplan–Meier survival curves and logistic regression to assess gender-related differences in outcomes. Results: Men had worse lung function at diagnosis (FVC 74.9 ± 18.5 vs. 87.2 ± 20.1% of pred.; p < 0.001) and a higher smoking prevalence (74% vs. 30%; p < 0.001). Women had better survival (51.2 vs. 40.8 ± 19.2 months; p = 0.005) despite more frequent biopsy use (36% vs. 17%; p = 0.013). Women tolerated longer therapy better (p = 0.001). No differences were found between patients receiving reduced antifibrotic dosing and those receiving full dosing. Conclusions: Gender has a significant impact on IPF outcomes, with women demonstrating better survival and tolerance to long-term therapy. In contrast, reducing antifibrotic treatment does not appear to significantly affect survival outcomes. These findings underscore the need for future research on gender-specific management approaches. Full article
(This article belongs to the Special Issue Advances in Pulmonary Fibrosis)
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