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Pulmonary Fibrosis: Therapeutic and Management Strategies
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Pulmonary Fibrosis: Therapeutic and Management Strategies

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

Deadline for manuscript submissions: closed (15 July 2024) | Viewed by 8528

Special Issue Editor

Prof. Dr. Martina Bonifazi

E-Mail Website
Guest Editor
1. Department of Biomedical Sciences and Public Health, Marche Polytechnic University, 60020 Ancona, Italy
2. Respiratory Diseases Unit, Azienda Ospedaliero-Universitaria “Ospedali Riuniti”, 60126 Ancona, Italy
Interests: interstitial lung diseases; epidemiology; interventional pulmonology
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

The term “interstitial lung diseases” (ILDs) includes a wide spectrum of heterogeneous entities with different prognoses, as well as treatment options. Lung fibrosis is the late stage of many chronic, systemic or localized lung diseases, characterized by immune-mediated inflammation, such as hypersensitivity pneumonitis (HP) and autoimmune conditions, or via epithelial-driven dysfunction, such as idiopathic pulmonary fibrosis (IPF). Although historically thought of as rare diseases, the latest epidemiologic data show an increasing trend of incidence and prevalence of these diseases worldwide, reflecting both changes in environmental exposure and the higher awareness of these entities among clinicians.

Over the last decade, there has been several advances in our comprehension of pathogenic mechanisms, as well as of predictors of clinical evolution, due to the adoption of innovative diagnostic technologies, such as cryobiopsy and molecular classifiers, and several potential clinical and biological biomarkers are currently under investigation for diagnostic and therapeutic purposes.

The management of fibrosis lung diseases is highly complex and necessarily requires effective integration between different specialists and the support of innovative technologies, including telemedicine.

The aim of this Special Issue is to highlight the recent advances in the therapeutic and management strategies in this field, with a particular focus on progressive fibrosis phenotype.

Prof. Dr. Martina Bonifazi
Guest Editor

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Keywords

  • pulmonary fibrosis
  • management
  • therapy
  • biomarkers
  • progressive phenotype

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Published Papers (6 papers)

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Research

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13 pages, 4017 KiB  
Article
Differences Between Patients with Probable UIP and Definite UIP on HRCT in Idiopathic Pulmonary Fibrosis: A Real-World Cohort Study
by Tao Chen, Cheng-Sheng Yin, Ping Wang, Qiu-Hong Li, Chi Shao, Hui Huang, Lan Song, Wei-Hong Zhang and Zuo-Jun Xu
J. Clin. Med. 2024, 13(23), 7170; https://doi.org/10.3390/jcm13237170 - 26 Nov 2024
Viewed by 246
Abstract
Background: Both a probable usual interstitial pneumonia (UIP) pattern (p-UIP) and a definite UIP pattern (d-UIP) on high-resolution computed tomography (HRCT) are sufficient to establish a diagnosis of idiopathic pulmonary fibrosis (IPF) without the need for a surgical lung biopsy, according to the [...] Read more.
Background: Both a probable usual interstitial pneumonia (UIP) pattern (p-UIP) and a definite UIP pattern (d-UIP) on high-resolution computed tomography (HRCT) are sufficient to establish a diagnosis of idiopathic pulmonary fibrosis (IPF) without the need for a surgical lung biopsy, according to the 2022 IPF guidelines. However, it remains unknown whether patients with p-UIP and d-UIP have similar disease behaviors and clinical courses. Material and Methods: We retrospectively collected clinical data of patients with IPF and divided the patients into two groups according to their HRCT patterns: a p-UIP group and a d-UIP group. The baseline characteristics, survival rates, and pulmonary function tests were compared between the two groups. The risk factors for mortality were determined by Cox regression in p-UIP and d-UIP separately. Results: There were 304 patients in the p-UIP group and 480 patients in the d-UIP group. Patients in the d-UIP group were more likely to have smoking histories (p < 0.001) and had lower baseline FVC% (74% vs. 77%, p = 0.021) and DLCO% (50% vs. 58%, p < 0.001). Survival rates were higher in p-UIP compared with d-UIP (p = 0.004). There were no differences in changes in FVC% or DLCO% between the two groups. Baseline DLCO% was the only independent risk factor for mortality in p-UIP. Baseline FVC% was independently associated with mortality in d-UIP. Symptom of cough was a risk factor for disease progression (OR = 1.2, p = 0.002) in p-UIP, while symptom of dyspnea might be associated with disease progression in d-UIP (OR = 2.7, p = 0.065). Male patients (OR = 1.88, p = 0.002) with a smoking history (OR = 1.16, p = 0.002) were at higher risk of developing d-UIP. Conclusions: We observed the different disease trajectories between p-UIP and d-UIP. P-UIP on HRCT might identify a subgroup of IPF patients who are in the early stage with a better prognosis. Full article
(This article belongs to the Special Issue Pulmonary Fibrosis: Therapeutic and Management Strategies)
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11 pages, 448 KiB  
Article
The Prognostic Role of Advanced Lung Cancer Inflammation Index in Patients with Idiopathic Pulmonary Fibrosis
by Fulsen Bozkuş and Olgun Keskin
J. Clin. Med. 2024, 13(19), 5874; https://doi.org/10.3390/jcm13195874 - 2 Oct 2024
Viewed by 724
Abstract
Background: The advanced lung cancer inflammation index (ALI) is an innovative and thorough measure designed to assess both inflammation and nutritional status. It includes parameters such as albumin, body mass index (BMI), and the neutrophil-to-lymphocyte ratio (NLR). This research seeks to evaluate [...] Read more.
Background: The advanced lung cancer inflammation index (ALI) is an innovative and thorough measure designed to assess both inflammation and nutritional status. It includes parameters such as albumin, body mass index (BMI), and the neutrophil-to-lymphocyte ratio (NLR). This research seeks to evaluate the prognosis of idiopathic pulmonary fibrosis (IPF) patients by integrating both inflammation and nutritional status, distinguishing it from conventional inflammation biomarkers. Methods: This study included 102 patients with IPF. Clinical data were extracted from the patients’ medical records. NLR and ALI scores were calculated based on data collected at the initiation of antifibrotic treatment using the following formulas: Neut/Lym for NLR and albumin × BMI/NLR for ALI. Results: ALI values were assessed across various IPF patient subgroups based on gender–age–physiology (GAP) stages (1, 2, and 3), forced vital capacity (FVC) (median split: <70% vs. ≥70%), diffusing capacity for carbon monoxide (DLCO) (<51% vs. ≥51%), 6-Minute Walk Test (6MWT) (<350 vs. ≥350), and the Charlson comorbidity index (CCI) (≤1 vs. >1). Significant differences in ALI were observed with respect to GAP stages, FVC, DLCO, and 6MWT categories (p = 0.000 for all), but not for CCI categories (p = 0.233). Receiver operating characteristic (ROC) curve analysis revealed that ALI had a sensitivity of 63.6% and a specificity of 98.9% at a threshold of 11.2 (AUC = 0.945, 95% CI 0.892–0.998, p < 0.000). Conclusions: Our findings indicate that ALI levels are significantly associated with disease severity and mortality in IPF patients. Full article
(This article belongs to the Special Issue Pulmonary Fibrosis: Therapeutic and Management Strategies)
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10 pages, 799 KiB  
Article
Understanding Patterns of Adherence to Antifibrotic Treatment in Idiopathic Pulmonary Fibrosis: Insights from an Italian Prospective Cohort Study
by Marica Iommi, Francesca Gonnelli, Martina Bonifazi, Andrea Faragalli, Federico Mei, Marco Pompili, Flavia Carle and Rosaria Gesuita
J. Clin. Med. 2024, 13(9), 2727; https://doi.org/10.3390/jcm13092727 - 6 May 2024
Cited by 3 | Viewed by 1483
Abstract
Background: Pirfenidone and Nintedanib have significantly improved the prognosis of patients with idiopathic pulmonary fibrosis (IPF), reducing mortality risk and exacerbations. This study aimed to analyze antifibrotic treatment utilization and its association with clinical outcomes (i.e., acute exacerbation or death) during 2014–2021 [...] Read more.
Background: Pirfenidone and Nintedanib have significantly improved the prognosis of patients with idiopathic pulmonary fibrosis (IPF), reducing mortality risk and exacerbations. This study aimed to analyze antifibrotic treatment utilization and its association with clinical outcomes (i.e., acute exacerbation or death) during 2014–2021 in newly diagnosed IPF patients, using Healthcare Utilization Databases of the Marche Region, Italy. Methods: The first 12-month adherence to antifibrotic was estimated using the Proportion of Days Covered (PDC), defining adherence as PDC ≥ 75%. State Sequence Analysis over the initial 52 weeks of treatment was used to identify adherence patterns. The role of adherence patterns on acute exacerbations/death, adjusted by demographic, clinical features, and monthly adherence after the 52-week period (time-dependent variable), was assessed with Cox regression. Results: Among 667 new IPF cases, 296 received antifibrotic prescriptions, with 62.8% being adherent in the first year. Three antifibrotic utilization patterns emerged—high adherence (37.2%), medium adherence (42.5%), and low adherence (20.3%)—with median PDCs of 95.3%, 79.5%, and 18.6%, respectively. These patterns did not directly influence three-year mortality/exacerbation probability, but sustained adherence reduced risk over time. Conclusions: Good adherence was observed in in this population-based study, emphasizing the importance of continuous antifibrotics therapy over time to mitigate adverse outcomes. Full article
(This article belongs to the Special Issue Pulmonary Fibrosis: Therapeutic and Management Strategies)
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18 pages, 2914 KiB  
Article
A Real-World Multicenter Retrospective Observational Study on Polish Experience with Nintedanib Therapy in Patients with Idiopathic Pulmonary Fibrosis: The PolExNIB Study
by Sebastian Majewski, Adam J. Białas, Adam Barczyk, Halina Batura-Gabryel, Małgorzata Buchczyk, Anna Doboszyńska, Katarzyna Górska, Luiza Grabowska-Skudlarz, Hanna Jagielska-Len, Agnieszka Jarzemska, Ewa Jassem, Dariusz Jastrzębski, Aleksander Kania, Marek Koprowski, Michał Krawczyk, Rafał Krenke, Katarzyna Lewandowska, Barbara Mackiewicz, Magdalena M. Martusewicz-Boros, Janusz Milanowski, Małgorzata Noceń-Piskorowska, Agata Nowicka, Kazimierz Roszkowski-Śliż, Alicja Siemińska, Krzysztof Sładek, Małgorzata Sobiecka, Tomasz Stachura, Małgorzata Tomczak, Witold Tomkowski, Marzena Trzaska-Sobczak, Dariusz Ziora, Beata Żołnowska and Wojciech J. Piotrowskiadd Show full author list remove Hide full author list
J. Clin. Med. 2023, 12(14), 4635; https://doi.org/10.3390/jcm12144635 - 12 Jul 2023
Cited by 1 | Viewed by 1962
Abstract
Nintedanib is a disease-modifying agent licensed for the treatment of IPF. Data on Polish experience with nintedanib in IPF are lacking. The present study aimed to describe the safety and efficacy profiles of nintedanib in a large real-world cohort of Polish patients with [...] Read more.
Nintedanib is a disease-modifying agent licensed for the treatment of IPF. Data on Polish experience with nintedanib in IPF are lacking. The present study aimed to describe the safety and efficacy profiles of nintedanib in a large real-world cohort of Polish patients with IPF. This was a multicenter, retrospective, observational study of IPF patients treated with nintedanib between March 2018 and October 2021. Data collection included baseline clinical characteristics, results of pulmonary function tests (PFTs), and a six-minute walk test (6MWT). Longitudinal data on PFTs, 6MWT, adverse drug reactions (ADRs), and treatment persistence were also retrieved. A total of 501 patients (70% male) with a median age of 70.9 years (IQR 65–75.7) were included in this study. Patients were followed on treatment for a median of 15 months (7–25.5). The majority of patients (66.7%) were treated with the full recommended dose of nintedanib and 33.3% of patients were treated with a reduced dose of a drug. Intermittent dose reductions or drug interruptions were needed in 20% of patients. Over up to 3 years of follow-up, pulmonary function remained largely stable with the minority experiencing disease progression. The most frequent ADRs included diarrhea (45.3%), decreased appetite (29.9%), abdominal discomfort (29.5%), weight loss (32.1%), nausea (20.8%), fatigue (19.2%), increased liver aminotransferases (15.4%), and vomiting (8.2%). A total of 203 patients (40.5%) discontinued nintedanib treatment due to diverse reasons including ADRs (10.2%), death (11.6%), disease progression (4.6%), patient’s request (6.6%), and neoplastic disease (2.2%). This real-world study of a large cohort of Polish patients with IPF demonstrates that nintedanib therapy is safe, and is associated with acceptable tolerance and disease stabilization. These data support the findings of previously conducted clinical trials and observational studies on the safety and efficacy profiles of nintedanib in IPF. Full article
(This article belongs to the Special Issue Pulmonary Fibrosis: Therapeutic and Management Strategies)
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Review

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16 pages, 828 KiB  
Review
The Beneficial Impact of Pulmonary Rehabilitation in Idiopathic Pulmonary Fibrosis: A Review of the Current Literature
by Stefano Sanduzzi Zamparelli, Carmen Lombardi, Claudio Candia, Paola Rebecca Iovine, Gaetano Rea, Michele Vitacca, Pasquale Ambrosino, Marialuisa Bocchino and Mauro Maniscalco
J. Clin. Med. 2024, 13(7), 2026; https://doi.org/10.3390/jcm13072026 - 30 Mar 2024
Viewed by 2346
Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic and irreversible fibrotic disease whose natural history is characterised by a progressive worsening of the pulmonary function, exertional dyspnoea, exercise intolerance, reduced physical activity, and health-related quality of life (HRQOL) impairment. Pulmonary rehabilitation (PR) is a [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is a chronic and irreversible fibrotic disease whose natural history is characterised by a progressive worsening of the pulmonary function, exertional dyspnoea, exercise intolerance, reduced physical activity, and health-related quality of life (HRQOL) impairment. Pulmonary rehabilitation (PR) is a comprehensive, multi-disciplinary programme that uses a combination of strength training, teaching, counselling, and behaviour modification techniques to reduce symptoms and optimise functional capacity in patients with chronic lung disease. Based on the well-documented effectiveness of PR in chronic obstructive pulmonary disease (COPD), over the years supportive evidence of its benefits for other respiratory diseases has been emerging. Although the latest rehabilitation guidelines recognised PR’s efficacy for interstitial lung disease (ILD) and IPF in particular, this comprehensive approach remains underused and under-resourced. In this review, we will discuss the advantages and beneficial effects of PR on IPF, analysing its impact on exercise capacity, disease-related symptoms, cardiovascular outcomes, body composition, and HRQOL. Full article
(This article belongs to the Special Issue Pulmonary Fibrosis: Therapeutic and Management Strategies)
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12 pages, 2278 KiB  
Review
Spontaneous Ventilation Thoracoscopic Lung Biopsy in Undetermined Interstitial Lung Disease: Systematic Review and Meta-Analysis
by Alexandro Patirelis, Stefano Elia, Benedetto Cristino, Ermanno Puxeddu, Francesco Cavalli, Paola Rogliani and Eugenio Pompeo
J. Clin. Med. 2024, 13(2), 374; https://doi.org/10.3390/jcm13020374 - 10 Jan 2024
Viewed by 1128
Abstract
Thoracoscopic surgical biopsy has shown excellent histological characterization of undetermined interstitial lung diseases, although the morbidity rates reported are not negligible. In delicate patients, interstitial lung disease and restrictive ventilatory impairment morbidity are thought to be due at least in part to tracheal [...] Read more.
Thoracoscopic surgical biopsy has shown excellent histological characterization of undetermined interstitial lung diseases, although the morbidity rates reported are not negligible. In delicate patients, interstitial lung disease and restrictive ventilatory impairment morbidity are thought to be due at least in part to tracheal intubation with single-lung mechanical ventilation; therefore, spontaneous ventilation thoracoscopic lung biopsy (SVTLB) has been proposed as a potentially less invasive surgical option. This systematic review summarizes the results of SVTLB, focusing on diagnostic yield and operative morbidity. A systematic search for original studies regarding SVTLB published between 2010 to 2023 was performed. In addition, articles comparing SVTLB to mechanical ventilation thoracoscopic lung biopsy (MVTLB) were selected for a meta-analysis. Overall, 13 studies (two before 2017 and eleven between 2018 and 2023) entailing 675 patients were included. Diagnostic yield ranged from 84.6% to 100%. There were 64 (9.5%) complications, most of which were minor. There was no 30-day operative mortality. When comparing SVTLB to MVTLB, the former group showed a significantly lower risk of complications (p < 0.001), whereas no differences were found in diagnostic accuracy. The results of this review suggest that SVTLB is being increasingly adopted worldwide and has proven to be a safe procedure with excellent diagnostic accuracy. Full article
(This article belongs to the Special Issue Pulmonary Fibrosis: Therapeutic and Management Strategies)
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