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Special Issue "Idiopathic Pulmonary Fibrosis Research"

A special issue of Medicina (ISSN 1010-660X).

Deadline for manuscript submissions: closed (30 June 2019).

Special Issue Editor

Guest Editor
Prof. Dr. Marco Confalonieri

Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste, Italy
Website | E-Mail
Interests: respiratory diseases; pulmonary fibrosis; critical care; respiratory failure; lung regeneration

Special Issue Information

Dear Colleagues,

Idiopathic pulmonary fibrosis (IPF) is a spontaneously occurring, specific and progressive form of chronic fibrosing interstitial pneumonia associated with a poor prognosis: Despite the introduction of the newest antifibrotic drugs, Pirfenidone and Nintedanib, the median survival of patients with IPF still amounts to less than 5 years. Approximately 10-20% of patients have an acute exacerbation every year, which increases the burden of the disease; furthermore, IPF is associated with a higher risk of comorbidities such as lung cancer, pulmonary hypertension and venous thromboembolism.

Prevalence is highly variable worldwide and appears to be increasing, although it is still not clear whether this reflects an increased ability in recognition or a true increase in incidence. It is now emerging that IPF pathogenesis relies on cycles of injury and dysregulated repair, and a number of familial cases, associated genetic defects, and other risk factors have also been described; however, despite an improvement in the understanding of the pathobiological mechanisms involved in the disease, it still represents a scientific and clinical challenge.

When IPF is suspected, diagnosis is usually supported by a typical interstitial pneumonia pattern on high-resolution CT or thoracoscopic lung biopsy, while further data are needed to recommend the use of molecular approaches as adjuvants to diagnosis in clinical practice.

Given the relevance of IPF in the fields of respiratory medicine and research, the journal Medicina is launching this Special Issue.

We are pleased to invite you and your co-workers to submit your articles reporting on the topic. Reviews or original articles focusing on the biochemical and molecular aspects underlying the pathobiology of IPF, as well as articles providing an up-to-date overview of the use of circulating/tissue biomarkers in early diagnosis and management, are particularly welcome. We would also like to invite you to submit review articles dealing with epidemiological aspects, prevention strategies targeting newly discovered risk factors, and recent advances in therapeutics with special regards to individualized approaches, efficacy and safety profile of currently available therapies, real-world studies included. Finally, we kindly invite you to submit your work or review data concerning the treatment of acute exacerbations and prognosis after lung transplantation.

Prof. Dr. Marco Confalonieri
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 papers will be 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 1500 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

  • Usual Interstitial pneumonia
  • Idiopathic pulmonary fibrosis
  • Epithelial cells
  • Honeycombing
  • Pirfenidone
  • Nintedanib
  • Real-world IPF treatment
  • Respiratory failure
  • Cell cycle
  • Lung regeneration
  • Diagnostic algorithms
  • Biological markers
  • Prognosis
  • Epithelial mesenchymal transition
  • Lung biopsy
  • Acute exacerbations
  • Lung transplantation

Published Papers (7 papers)

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Research

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Open AccessArticle
Possible Serological Markers to Predict Mortality in Acute Exacerbation of Idiopathic Pulmonary Fibrosis
Medicina 2019, 55(5), 132; https://doi.org/10.3390/medicina55050132
Received: 28 February 2019 / Revised: 10 May 2019 / Accepted: 10 May 2019 / Published: 13 May 2019
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Abstract
Background and objectives: Idiopathic pulmonary fibrosis (IPF) has a particularly poor prognosis, and most IPF-related deaths are due to acute exacerbation (AE) of this condition. Few reports about biomarkers to predict prognosis of AE-IPF have been published since the release of the new [...] Read more.
Background and objectives: Idiopathic pulmonary fibrosis (IPF) has a particularly poor prognosis, and most IPF-related deaths are due to acute exacerbation (AE) of this condition. Few reports about biomarkers to predict prognosis of AE-IPF have been published since the release of the new AE-IPF criteria in 2016. The present study investigated relationships between serological markers and in-hospital mortality after the onset of AE-IPF. Methods: Demographic, serological, and imaging data from patients hospitalized at the Maebashi Red Cross Hospital (Gunma, Japan) between 1 January 2013, and 31 December 2017, were retrospectively reviewed. Subjects fulfilling the diagnostic criteria for AE-IPF were divided into those who survived or died; statistical analysis of risk factors was performed using data from these two groups. Results: Diagnostic criteria for AE-IPF were fulfilled by 84 patients (59 males (70.2%)), with a median age of 78 years (range, 56–95 years). IPF was diagnosed before hospitalization in 50 (59.5%) patients and 38 (45.2%) died in hospital. Among the serological markers at hospitalization in the deceased group, C-reactive protein (CRP) was significantly higher than in the survivor group (p = 0.002), while total serum protein (p = 0.031), albumin (p = 0.047) and total cholesterol (p = 0.039) were significantly lower. Cox hazard analysis of factors predicting mortality, corrected for age, sex and BMI, revealed the following: CRP (hazard ratio (HR) 1.080 (95% confidence interval (CI) 1.022–1.141); p = 0.006), LDH (HR 1.003 (95% CI 1.000–1.006); p = 0.037), and total cholesterol (HR 0.985 (95% CI 0.972–0.997); p = 0.018). Conclusions: Our data suggest that CRP, LDH, and total cholesterol may be biomarkers predicting mortality in patients with AE-IPF. However, only prospective controlled studies can confirm or not our observation as a generalizable one. Full article
(This article belongs to the Special Issue Idiopathic Pulmonary Fibrosis Research)
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Review

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Open AccessReview
Therapeutic Role of Recombinant Human Soluble Thrombomodulin for Acute Exacerbation of Idiopathic Pulmonary Fibrosis
Medicina 2019, 55(5), 172; https://doi.org/10.3390/medicina55050172
Received: 5 February 2019 / Revised: 11 March 2019 / Accepted: 15 May 2019 / Published: 20 May 2019
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Abstract
Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an acute respiratory worsening of unidentifiable cause that sometimes develops during the clinical course of IPF. Although the incidence of AE-IPF is not high, prognosis is poor. The pathogenesis of AE-IPF is not well understood; [...] Read more.
Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an acute respiratory worsening of unidentifiable cause that sometimes develops during the clinical course of IPF. Although the incidence of AE-IPF is not high, prognosis is poor. The pathogenesis of AE-IPF is not well understood; however, evidence suggests that coagulation abnormalities and inflammation are involved. Thrombomodulin is a transmembranous glycoprotein found on the cell surface of vascular endothelial cells. Thrombomodulin combines with thrombin, regulates coagulation/fibrinolysis balance, and has a pivotal role in suppressing excess inflammation through its inhibition of high-mobility group box 1 protein and the complement system. Thus, thrombomodulin might be effective in the treatment of AE-IPF, and we and other groups found that recombinant human soluble thrombomodulin improved survival in patients with AE-IPF. This review summarizes the existing evidence and considers the therapeutic role of thrombomodulin in AE-IPF. Full article
(This article belongs to the Special Issue Idiopathic Pulmonary Fibrosis Research)
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Open AccessReview
Epithelial–Mesenchymal Transition in the Pathogenesis of Idiopathic Pulmonary Fibrosis
Received: 30 December 2018 / Revised: 21 February 2019 / Accepted: 26 March 2019 / Published: 28 March 2019
Cited by 3 | PDF Full-text (859 KB) | HTML Full-text | XML Full-text
Abstract
Idiopathic pulmonary fibrosis (IPF) is a serious disease of the lung, which leads to extensive parenchymal scarring and death from respiratory failure. The most accepted hypothesis for IPF pathogenesis relies on the inability of the alveolar epithelium to regenerate after injury. Alveolar epithelial [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is a serious disease of the lung, which leads to extensive parenchymal scarring and death from respiratory failure. The most accepted hypothesis for IPF pathogenesis relies on the inability of the alveolar epithelium to regenerate after injury. Alveolar epithelial cells become apoptotic and rare, fibroblasts/myofibroblasts accumulate and extracellular matrix (ECM) is deposited in response to the aberrant activation of several pathways that are physiologically implicated in alveologenesis and repair but also favor the creation of excessive fibrosis via different mechanisms, including epithelial–mesenchymal transition (EMT). EMT is a pathophysiological process in which epithelial cells lose part of their characteristics and markers, while gaining mesenchymal ones. A role for EMT in the pathogenesis of IPF has been widely hypothesized and indirectly demonstrated; however, precise definition of its mechanisms and relevance has been hindered by the lack of a reliable animal model and needs further studies. The overall available evidence conceptualizes EMT as an alternative cell and tissue normal regeneration, which could open the way to novel diagnostic and prognostic biomarkers, as well as to more effective treatment options. Full article
(This article belongs to the Special Issue Idiopathic Pulmonary Fibrosis Research)
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Open AccessReview
Acute Exacerbation of Idiopathic Pulmonary Fibrosis
Received: 17 December 2018 / Revised: 18 February 2019 / Accepted: 14 March 2019 / Published: 16 March 2019
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Abstract
Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonia. Idiopathic pulmonary fibrosis is often seen in elderly men who smoke. A diagnosis of IPF is based on a combination of a detailed clinical history, specific physical examination, laboratory findings, [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonia. Idiopathic pulmonary fibrosis is often seen in elderly men who smoke. A diagnosis of IPF is based on a combination of a detailed clinical history, specific physical examination, laboratory findings, pulmonary function tests, high-resolution computed tomography (HRCT) of the chest, and histopathology. Idiopathic pulmonary fibrosis has a heterogeneous clinical course, from an asymptomatic stable state to progressive respiratory failure or acute exacerbation (AE). Acute exacerbation of IPF has several important differential diagnoses, such as heart failure and volume overload. The International Working Group project proposed new criteria for defining AE of IPF in 2016, which divides it into triggered and idiopathic AE. On the basis of these criteria, physicians can detect AE of IPF more easily. The recent international IPF guidelines emphasized the utility of chest HRCT. In addition, two antifibrotic agents have become available. We should focus on both the management and prevention of AE. The diagnostic process, laboratory findings, typical chest imaging, management, and prognosis of AE are comprehensively reviewed in this article. Full article
(This article belongs to the Special Issue Idiopathic Pulmonary Fibrosis Research)
Open AccessReview
The Role of Occupational and Environmental Exposures in the Pathogenesis of Idiopathic Pulmonary Fibrosis: A Narrative Literature Review
Medicina 2018, 54(6), 108; https://doi.org/10.3390/medicina54060108
Received: 19 November 2018 / Accepted: 5 December 2018 / Published: 10 December 2018
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Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease characterised by a progressive and irreversible decline in lung function, which is associated with poor long-term survival. The pathogenesis of IPF is incompletely understood. An accumulating body of evidence, obtained over the past [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease characterised by a progressive and irreversible decline in lung function, which is associated with poor long-term survival. The pathogenesis of IPF is incompletely understood. An accumulating body of evidence, obtained over the past three decades, suggests that occupational and environmental exposures may play a role in the development of IPF. This narrative literature review aims to summarise current understanding and the areas of ongoing research into the role of occupational and environmental exposures in the pathogenesis of IPF. Full article
(This article belongs to the Special Issue Idiopathic Pulmonary Fibrosis Research)

Other

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Open AccessCase Report
Pirfenidone Improves Familial Idiopathic Pulmonary Fibrosis without Affecting Serum Periostin Levels
Medicina 2019, 55(5), 161; https://doi.org/10.3390/medicina55050161
Received: 21 February 2019 / Revised: 16 May 2019 / Accepted: 16 May 2019 / Published: 17 May 2019
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Abstract
Background: Antifibrotic agents have been approved for the treatment of idiopathic pulmonary fibrosis (IPF). However, the efficacy of these drugs in the treatment of familial IPF (FIPF) has not been previously reported. Case presentation: We report the case of a 77-year-old man with [...] Read more.
Background: Antifibrotic agents have been approved for the treatment of idiopathic pulmonary fibrosis (IPF). However, the efficacy of these drugs in the treatment of familial IPF (FIPF) has not been previously reported. Case presentation: We report the case of a 77-year-old man with FIPF, successfully treated with pirfenidone. His uncle died due to IPF, and his niece was diagnosed with the disease. He had worsening dyspnea two months prior to admission to our hospital. Upon admission, he had desaturation when exercising and broad interstitial pneumonia. Administration of pirfenidone improved his dyspnea, desaturation, and the reticular shadow on his chest radiograph. Increased fibrotic marker levels KL-6 and SP-D were also normalized in six months; treatment had no effect on his serum periostin level. Pirfenidone has been effective for over two years. Conclusion: Antifibrotic agents such as pirfenidone may be useful for the management of FIPF, as well as cases of sporadic IPF. Full article
(This article belongs to the Special Issue Idiopathic Pulmonary Fibrosis Research)
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Open AccessCase Report
An Atypical Case of Idiopathic Pulmonary Fibrosis in a Patient from Africa
Received: 11 February 2019 / Accepted: 11 March 2019 / Published: 14 March 2019
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Abstract
A 39 years old African man presented with fatigue, loss of weight and night sweats; radiology showed a possible usual interstitial pneumonia pattern. The patient missed follow-up visits, and presented again after 3 years with productive cough and general illness. Pulmonary function tests [...] Read more.
A 39 years old African man presented with fatigue, loss of weight and night sweats; radiology showed a possible usual interstitial pneumonia pattern. The patient missed follow-up visits, and presented again after 3 years with productive cough and general illness. Pulmonary function tests showed a decline of FVC compared to a previous investigation. The CT scans showed progression of the interstitial lung disease, and a multidisciplinary conference recommended to proceed with a surgical lung biopsy. Histopathology showed an atypical pattern, with bronchiolar metaplasia. A new multidisciplinary conference made a diagnosis of IPF, and the patient was treated with antifibrotic drugs with a good effect, reaching stability of lung function. This case report highlights the need to improve knowledge and to better characterize rare pulmonary diseases, and especially IPF, among African patients. Full article
(This article belongs to the Special Issue Idiopathic Pulmonary Fibrosis Research)
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