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Idiopathic Pulmonary Fibrosis Research

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A special issue of Medicina (ISSN 1648-9144).

Deadline for manuscript submissions: closed (30 June 2019) | Viewed by 45181

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Special Issue Editor

Prof. Dr. Marco Confalonieri

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Guest Editor

Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste, Italy
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

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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 (11 papers)

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Research

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11 pages, 869 KiB

Open AccessArticle

Lung Cryobiopsy for the Diagnosis of Interstitial Lung Diseases: A Series Contribution to a Debated Procedure

by Sergio Harari, Francesca Cereda, Federico Pane, Alberto Cavazza, Nikolaos Papanikolaou, Giuseppe Pelosi, Monica Scarioni, Elisabetta Uslenghi, Maurizio Zompatori and Antonella Caminati

Medicina 2019, 55(9), 606; https://doi.org/10.3390/medicina55090606 - 19 Sep 2019

Cited by 16 | Viewed by 2481

Abstract

Introduction: Transbronchial cryobiopsy is an alternative to surgical biopsy for the diagnosis of fibrosing interstitial lung diseases, although the role of this relatively new method is rather controversial. Aim of this study is to evaluate the diagnostic performance and the safety of transbronchial cryobiopsy in patients with fibrosing interstitial lung disease. Materials and methods: The population in this study included patients with interstitial lung diseases who underwent cryobiopsy from May 2015 to May 2018 at the Division of Pneumology of San Giuseppe Hospital in Milan and who were retrospectively studied. All cryobiopsy procedures were performed under fluoroscopic guidance using a flexible video bronchoscope and an endobronchial blocking system in the operating room with patients under general anaesthesia. The diagnostic performance and safety of the procedure were assessed. The main complications evaluated were endobronchial bleeding and pneumothorax. All cases were studied with a multidisciplinary approach, before and after cryobiopsy. Results: Seventy-three patients were admitted to this study. A specific diagnosis was reached in 64 cases, with a diagnostic sensitivity of 88%; 5 cases (7%) were considered inadequate, 4 cases (5%) were found to be non-diagnostic. Only one major bleeding event occurred (1.4%), while 14 patients (19%) experienced mild/moderate bleeding events while undergoing bronchoscopy; 8 cases of pneumothorax (10.9%) were reported, of which 2 (2.7%) required surgical drainage. Conclusions: When performed under safe conditions and in an experienced center, cryobiopsy is a procedure with limited complications having a high diagnostic yield in fibrotic interstitial lung disease. Full article

(This article belongs to the Special Issue Idiopathic Pulmonary Fibrosis Research)

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11 pages, 635 KiB

Open AccessArticle

Possible Serological Markers to Predict Mortality in Acute Exacerbation of Idiopathic Pulmonary Fibrosis

by Yoshimasa Hachisu, Keisuke Murata, Kousuke Takei, Takuma Tsuchiya, Hiroaki Tsurumaki, Yasuhiko Koga, Takeo Horie, Atsushi Takise and Takeshi Hisada

Medicina 2019, 55(5), 132; https://doi.org/10.3390/medicina55050132 - 13 May 2019

Cited by 17 | Viewed by 2729

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 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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11 pages, 658 KiB

Open AccessReview

Idiopathic Pulmonary Fibrosis and Lung Transplantation: When it is Feasible

by Elisabetta Balestro, Elisabetta Cocconcelli, Mariaenrica Tinè, Davide Biondini, Eleonora Faccioli, Marina Saetta and Federico Rea

Medicina 2019, 55(10), 702; https://doi.org/10.3390/medicina55100702 - 19 Oct 2019

Cited by 19 | Viewed by 4314

Abstract

Despite the availability of antifibrotic therapies, many patients with idiopathic pulmonary fibrosis (IPF) will progress to advanced disease and require lung transplantation. International guidelines for transplant referral and listing of patients with interstitial lung disease are not specific to those with IPF and were published before the widespread use of antifibrotic therapy. In this review, we discussed difficulties in decision-making when dealing with patients with IPF due to the wide variability in clinical course and life expectancy, as well as the acute deterioration associated with exacerbations. Indeed, the ideal timing for referral and listing for lung transplant remains challenging, and the acute deterioration might be influenced after transplant outcomes. Of note, patients with IPF are frequently affected by multimorbidity, thus a screening program for occurring conditions, such as coronary artery disease and pulmonary hypertension, before lung transplant listing is crucial to candidate selection, risk stratification, and optimal outcomes. Among several comorbidities, it is of extreme importance to highlight that the prevalence of lung cancer is increased amongst patients affected by IPF; therefore, candidates’ surveillance is critical to avoid organ allocation to unsuitable patients. For all these reasons, early referral and close longitudinal follow-up for potential lung transplant candidates are widely encouraged. Full article

(This article belongs to the Special Issue Idiopathic Pulmonary Fibrosis Research)

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12 pages, 3074 KiB

Open AccessReview

Imaging Review of the Lung Parenchymal Complications in Patients with IPF

by Elisa Baratella, Ilaria Fiorese, Cristina Marrocchio, Francesco Salton and Maria Assunta Cova

Medicina 2019, 55(10), 613; https://doi.org/10.3390/medicina55100613 - 20 Sep 2019

Cited by 7 | Viewed by 3446

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, pulmonary-limited, interstitial lung disease with a poor prognosis. This condition is characterized by different clinical scenarios, ranging from the most typical slow and progressive deterioration of symptoms to a rapid and abrupt decline of lung function. Rapid worsening of clinical course is due to superimposed complications and comorbidities that can develop in IPF patients, with a higher incidence rate compared to the general population. These conditions may require a different management of the patient and a therapy adjustment, and thus it is fundamental to recognize them. High Resolution Computed Tomography (HRCT) is sensitive, but not specific, in detecting these complications, and can evaluate the presence of radiological variations when previous examinations are available; it recognizes ground glass opacities or consolidation that can be related to a large spectrum of comorbidities, such as infection, lung cancer, or acute exacerbation. To reach the final diagnosis, a multidisciplinary discussion is required, particularly when the clinical context is related to imaging findings. Full article

(This article belongs to the Special Issue Idiopathic Pulmonary Fibrosis Research)

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13 pages, 639 KiB

Open AccessReview

Therapeutic Role of Recombinant Human Soluble Thrombomodulin for Acute Exacerbation of Idiopathic Pulmonary Fibrosis

by Takuma Isshiki, Susumu Sakamoto and Sakae Homma

Medicina 2019, 55(5), 172; https://doi.org/10.3390/medicina55050172 - 20 May 2019

Cited by 6 | Viewed by 2756

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; 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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8 pages, 859 KiB

Open AccessReview

Epithelial–Mesenchymal Transition in the Pathogenesis of Idiopathic Pulmonary Fibrosis

by Francesco Salton, Maria Concetta Volpe and Marco Confalonieri

Medicina 2019, 55(4), 83; https://doi.org/10.3390/medicina55040083 - 28 Mar 2019

Cited by 150 | Viewed by 8120

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 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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7 pages, 263 KiB

Open AccessReview

Acute Exacerbation of Idiopathic Pulmonary Fibrosis

by Tomoo Kishaba

Medicina 2019, 55(3), 70; https://doi.org/10.3390/medicina55030070 - 16 Mar 2019

Cited by 33 | Viewed by 7980

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, 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)

11 pages, 312 KiB

Open AccessReview

The Role of Occupational and Environmental Exposures in the Pathogenesis of Idiopathic Pulmonary Fibrosis: A Narrative Literature Review

by Samuel P. Trethewey and Gareth I. Walters

Medicina 2018, 54(6), 108; https://doi.org/10.3390/medicina54060108 - 10 Dec 2018

Cited by 27 | Viewed by 4486

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 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)

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10 pages, 652 KiB

Open AccessBrief Report

Choice of Methodology Impacts Outcome in Indirect Comparisons of Drugs for Idiopathic Pulmonary Fibrosis

by David A. Scott, Emma Loveman, Jill L. Colquitt and Katherine O’Reilly

Medicina 2019, 55(8), 443; https://doi.org/10.3390/medicina55080443 - 06 Aug 2019

Cited by 1 | Viewed by 2313

Abstract

Background and Objectives: Idiopathic pulmonary fibrosis (IPF) is a chronic condition leading to lung damage and deterioration in lung function. Following the availability of two new drugs, nintedanib and pirfenidone, a number of network meta-analyses (NMAs) of randomised controlled trials have been published which have conducted indirect comparisons on the two drugs. Differing recommendations from these studies are potentially confusing to clinicians and decision-makers. We aimed to systematically review published NMAs of IPF treatments, to compare their findings and summarise key recommendations. Materials and Methods: We systematically reviewed (PROSPERO: CRD42017072876) six eligible NMAs and investigated the differences in their findings with respect to key endpoints. We focused on differences in head-to-head comparisons between nintedanib and pirfenidone. Results: The NMAs were broadly consistent, with most differences being explained by model choice, endpoint definitions, inclusion of different studies, different follow-up durations, and access to unpublished data. A substantive difference remained, however, in the change from baseline forced vital capacity (FVC). One NMA favoured nintedanib, another found no statistical difference, whilst others did not conduct the analysis. These differences can be attributed to the choice of methodology, the use of the standardised mean difference (SMD) scale, and population heterogeneity. Conclusions: NMA methods facilitated the comparison of nintedanib and pirfenidone in the absence of a head-to-head trial. However, further work is needed to determine whether the trial populations are homogeneous and whether the SMD is appropriate in this population. Differences in patient characteristics may obscure the difference in treatment effects. To assist decision-makers, an exploration of efficacy in real-world populations may be prudent. Full article

(This article belongs to the Special Issue Idiopathic Pulmonary Fibrosis Research)

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6 pages, 2225 KiB

Open AccessCase Report

Pirfenidone Improves Familial Idiopathic Pulmonary Fibrosis without Affecting Serum Periostin Levels

by Yasuhiko Koga, Yoshimasa Hachisu, Hiroaki Tsurumaki, Masakiyo Yatomi, Kyoichi Kaira, Shoichiro Ohta, Junya Ono, Kenji Izuhara, Kunio Dobashi and Takeshi Hisada

Medicina 2019, 55(5), 161; https://doi.org/10.3390/medicina55050161 - 17 May 2019

Cited by 6 | Viewed by 2659

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 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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6 pages, 2684 KiB

Open AccessCase Report

An Atypical Case of Idiopathic Pulmonary Fibrosis in a Patient from Africa

by Ida Pesonen, Cristian Ortiz and Giovanni Ferrara

Medicina 2019, 55(3), 67; https://doi.org/10.3390/medicina55030067 - 14 Mar 2019

Viewed by 3071

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 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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