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Diagnostics
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Advances in the Diagnosis and Management of Interstitial Lung Disease
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Advances in the Diagnosis and Management of Interstitial Lung Disease

A special issue of Diagnostics (ISSN 2075-4418). This special issue belongs to the section "Pathology and Molecular Diagnostics".

Deadline for manuscript submissions: closed (29 February 2024) | Viewed by 12134

Special Issue Editors

Dr. Athena Gogali

E-Mail Website
Guest Editor
Consultant in Respiratory Medicine, Respiratory Medicine Department, University Hospital of Ioannina, Ioannina, Greece
Interests: Interstitial lung disease; severe asthma; bronchoscopy
Dr. Argyrios Tzouvelekis

E-Mail Website
Guest Editor
1. Associate Professor of Internal and Respiratory Medicine, Head Department of Respiratory Medicine, University of Patras, Patras, Greece
2. Associate Professor Adjunct, PCCSM, Yale School of Medicine, New Haven, CT, USA
Interests: interstitial lung disease; interventional pulmonology; severe asthma; lung cancer

Special Issue Information

Dear Colleagues,

Interstitial Lung Disease (ILD) includes a diverse spectrum of entities, which are characterized by various degrees of inflammation and fibrosis. The cardinal disease at the fibrotic end of this spectrum is Idiopathic Pulmonary Fibrosis (IPF), mainly due to its devastating nature. Nevertheless, it has been pointed out lately that a considerable proportion of patients with other underlying ILD diagnosis, either idiopathic or of known etiology, may have a similar-to-IPF clinical course, exhibiting a progressive phenotype. Thus, the new term "Progressive Pulmonary Fibrosis" has been introduced, lumping together different, in terms of pathology, conditions and leading to a new concept in treating these patients based on disease behavior. The accurate and prompt diagnosis of individual ILDs remains crucial, whereas the differential diagnosis is quite often challenging. Advances in bronchoscopy and radiology, along with new deep learning techniques and novel available data on genetics, molecular biomarkers and proteomics provide enhanced diagnostic and prognostic possibilities. Finally, evidence-based updated guidelines for the diagnosis and treatment of several ILDs as IPF, sarcoidosis, scleroderma, hypersensitivity pneumonitis, as well as criteria to define progressive fibrotic lung disease came recently to light, reflecting the increased interest in this field. This Special Issue on "Advances in the Diagnosis and Management of Interstitial Lung Disease" aims to cover all the new evidence on detection, diagnosis, prediction and therapeutic approach of ILDs through concise review papers from experts in the field. Relevant original research articles are welcome as well.

Dr. Athena Gogali
Dr. Argyrios Tzouvelekis
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. Diagnostics 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.

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Published Papers (5 papers)

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Research

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14 pages, 4454 KiB  
Article
Voxel-Wise Comparison of Co-Registered Quantitative CT and Hyperpolarised Gas Diffusion-Weighted MRI Measurements in IPF
by Ho-Fung Chan, Nicholas D. Weatherley, Alberto M. Biancardi, Christopher S. Johns, Bilal A. Tahir, Ronald A. Karwoski, Brian J. Bartholmai, Stephen M. Bianchi and Jim M. Wild
Diagnostics 2023, 13(23), 3497; https://doi.org/10.3390/diagnostics13233497 - 21 Nov 2023
Viewed by 1301
Abstract
The patterns of idiopathic pulmonary fibrosis (IPF) lung disease that directly correspond to elevated hyperpolarised gas diffusion-weighted (DW) MRI metrics are currently unknown. This study aims to develop a spatial co-registration framework for a voxel-wise comparison of hyperpolarised gas DW-MRI and CALIPER quantitative [...] Read more.
The patterns of idiopathic pulmonary fibrosis (IPF) lung disease that directly correspond to elevated hyperpolarised gas diffusion-weighted (DW) MRI metrics are currently unknown. This study aims to develop a spatial co-registration framework for a voxel-wise comparison of hyperpolarised gas DW-MRI and CALIPER quantitative CT patterns. Sixteen IPF patients underwent 3He DW-MRI and CT at baseline, and eleven patients had a 1-year follow-up DW-MRI. Six healthy volunteers underwent 129Xe DW-MRI at baseline only. Moreover, 3He DW-MRI was indirectly co-registered to CT via spatially aligned 3He ventilation and structural 1H MRI. A voxel-wise comparison of the overlapping 3He apparent diffusion coefficient (ADC) and mean acinar dimension (LmD) maps with CALIPER CT patterns was performed at baseline and after 1 year. The abnormal lung percentage classified with the LmD value, based on a healthy volunteer 129Xe LmD, and CALIPER was compared with a Bland–Altman analysis. The largest DW-MRI metrics were found in the regions classified as honeycombing, and longitudinal DW-MRI changes were observed in the baseline-classified reticular changes and ground-glass opacities regions. A mean bias of −15.3% (95% interval −56.8% to 26.2%) towards CALIPER was observed for the abnormal lung percentage. This suggests DW-MRI may detect microstructural changes in areas of the lung that are determined visibly and quantitatively normal by CT. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Interstitial Lung Disease)
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11 pages, 3707 KiB  
Article
Ultrasonographic Presentation and Anatomic Distribution of Lung Involvement in Patients with Rheumatoid Arthritis
by Marie Vermant, Alexandros Kalkanis, Tinne Goos, Heleen Cypers, Nico De Crem, Barbara Neerinckx, Veerle Taelman, Patrick Verschueren and Wim A. Wuyts
Diagnostics 2023, 13(18), 2986; https://doi.org/10.3390/diagnostics13182986 - 18 Sep 2023
Cited by 1 | Viewed by 1584
Abstract
Background: Rheumatoid arthritis (RA) is a chronic auto-immune disease, typically affecting the joints, which can also present with lung involvement (pleuritis, interstitial lung disease, pulmonary nodules, etc.). Lung ultrasound (LUS) is an upcoming tool in the detection of these pulmonary manifestations. Methods: We [...] Read more.
Background: Rheumatoid arthritis (RA) is a chronic auto-immune disease, typically affecting the joints, which can also present with lung involvement (pleuritis, interstitial lung disease, pulmonary nodules, etc.). Lung ultrasound (LUS) is an upcoming tool in the detection of these pulmonary manifestations. Methods: We performed a 72-window LUS in 75 patients presenting to the outpatient rheumatology clinic and describe the abnormalities (presence of B-lines (vertical comet-tail artefacts), pleural abnormalities, pleural effusions, and subpleural nodules) on lung ultrasound. We created a topological mapping of the number of B-lines per intercostal zone. Results: We observed pleural effusions, pleural abnormalities, and pleural nodules in, respectively, 1.3%, 45.3%, and 14% of patients. There were 35 (46.7%) patients who had less than 5 B-lines, 15 (20%) patients who had between 5 and 10 B-lines, 11 (14.6%) between 10 and 20, 10 (13.3%) between 20 and 50, 1 (1.3%) between 50 and 100, and 3 (4%) of patients who had more than 100 B-lines. Conclusions: LUS in patients with RA shows an array of abnormalities ranging from interstitial syndromes to pleural abnormalities, subpleural nodules, and pleural effusions. Hotspots for the presence of B-lines are situated bilaterally in the posterior subscapular regions, as well as the anterior right mid-clavicular region. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Interstitial Lung Disease)
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11 pages, 1102 KiB  
Article
Real-Life Diagnostic Performance of the Hypersensitivity Pneumonitis Guidelines: A Multicenter Cohort Study
by Ophir Freund, Yitzhac Hadad, Tamar Shalmon, Ori Wand, Sonia Schneer, Tal Moshe Perluk, Eyal Kleinhendler, Tzlil Hershko, Boaz Tiran, Galit Aviram, Evgeni Gershman, Yochai Adir, David Shitrit, Amir Bar-Shai and Avraham Unterman
Diagnostics 2023, 13(14), 2335; https://doi.org/10.3390/diagnostics13142335 - 11 Jul 2023
Cited by 2 | Viewed by 1726
Abstract
Hypersensitivity pneumonitis (HP) is a heterogeneous interstitial lung disease (ILD) that may be difficult to confidently diagnose. Recently, the 2020 ATS/JRS/ALAT HP diagnostic guidelines were published, yet data validating their performance in real-life settings are scarce. We aimed to assess the diagnostic performance [...] Read more.
Hypersensitivity pneumonitis (HP) is a heterogeneous interstitial lung disease (ILD) that may be difficult to confidently diagnose. Recently, the 2020 ATS/JRS/ALAT HP diagnostic guidelines were published, yet data validating their performance in real-life settings are scarce. We aimed to assess the diagnostic performance of the HP guidelines compared to the gold-standard multidisciplinary discussion (MDD). For this purpose, we included consecutive ILD patients that underwent diagnostic bronchoscopy between 2017 and 2020 in three large medical centers. Four diagnostic factors (antigen exposure history, chest computed tomography pattern, bronchoalveolar lavage lymphocyte count, and histology results) were used to assign guidelines-based HP diagnostic confidence levels for each patient. A sensitivity analysis was performed, with MDD diagnosis as the reference standard. Overall, 213 ILD patients were included, 45 (21%) with an MDD diagnosis of HP. The guidelines’ moderate (≥70%) confidence threshold produced optimal performance with 73% sensitivity for HP, 89% specificity, and a J-index of 0.62. The area under the receiver operating characteristic curve (AUC) for a correct guidelines-based diagnosis was 0.86. The guidelines had better performance for non-fibrotic than fibrotic HP (AUC 0.92 vs. 0.82). All diagnostic factors, except bronchoalveolar lavage lymphocyte count, were independent predictors for MDD diagnosis of HP in a multivariate analysis. In conclusion, the HP guidelines exhibited a good diagnostic performance compared to MDD diagnosis in real-life setting. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Interstitial Lung Disease)
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Review

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13 pages, 307 KiB  
Review
Pulmonary Hypertension in the Course of Interstitial Lung Diseases—A Personalised Approach Is Needed to Identify a Dominant Cause and Provide an Effective Therapy
by Aneta Kacprzak, Witold Tomkowski and Monika Szturmowicz
Diagnostics 2023, 13(14), 2354; https://doi.org/10.3390/diagnostics13142354 - 13 Jul 2023
Cited by 7 | Viewed by 2048
Abstract
The prevalence of pulmonary hypertension (PH) complicating interstitial lung diseases (ILDs) is 3.5–15% at an early stage, and up to 90% in ILD patients listed for lung transplantation. In addition, other types of PH may occur in patients with ILDs due to concomitant [...] Read more.
The prevalence of pulmonary hypertension (PH) complicating interstitial lung diseases (ILDs) is 3.5–15% at an early stage, and up to 90% in ILD patients listed for lung transplantation. In addition, other types of PH may occur in patients with ILDs due to concomitant conditions. Therefore, any significant PH occurring in the setting of ILD requires a proper differential workup. PH increases morbidity and mortality in ILDs. The pathomechanisms underlying PH due to ILD (PH-ILD) are not fully known, and there is no straightforward correlation between the presence or severity of PH-ILD and the severity of ILD. Severe PH in mild ILD without other explanatory causes constitutes a dilemma of differentiating between PH due to ILD and pulmonary arterial hypertension coexisting with ILDs. The heterogeneity and poor prognosis of patients with ILDs coexisting with PH necessitate an individualised approach to the management of this condition. This review presents recent advances in understanding and treatment options in PH-ILD. It also addresses practical issues, such as when to suspect and how to screen for PH in ILD, what are the indications for right heart catheterisation, and how to approach an individual ILD patient to determine the dominant PH cause and apply adequate management. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Interstitial Lung Disease)
13 pages, 1531 KiB  
Review
Recent Advances of Artificial Intelligence Applications in Interstitial Lung Diseases
by Konstantinos P. Exarchos, Georgia Gkrepi, Konstantinos Kostikas and Athena Gogali
Diagnostics 2023, 13(13), 2303; https://doi.org/10.3390/diagnostics13132303 - 6 Jul 2023
Cited by 11 | Viewed by 4499
Abstract
Interstitial lung diseases (ILDs) comprise a rather heterogeneous group of diseases varying in pathophysiology, presentation, epidemiology, diagnosis, treatment and prognosis. Even though they have been recognized for several years, there are still areas of research debate. In the majority of ILDs, imaging modalities [...] Read more.
Interstitial lung diseases (ILDs) comprise a rather heterogeneous group of diseases varying in pathophysiology, presentation, epidemiology, diagnosis, treatment and prognosis. Even though they have been recognized for several years, there are still areas of research debate. In the majority of ILDs, imaging modalities and especially high-resolution Computed Tomography (CT) scans have been the cornerstone in patient diagnostic approach and follow-up. The intricate nature of ILDs and the accompanying data have led to an increasing adoption of artificial intelligence (AI) techniques, primarily on imaging data but also in genetic data, spirometry and lung diffusion, among others. In this literature review, we describe the most prominent applications of AI in ILDs presented approximately within the last five years. We roughly stratify these studies in three categories, namely: (i) screening, (ii) diagnosis and classification, (iii) prognosis. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Interstitial Lung Disease)
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