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Diagnostics
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Clinical and Radiological Features of Interstitial Lung Diseases 2.0
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Clinical and Radiological Features of Interstitial Lung Diseases 2.0

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

Deadline for manuscript submissions: closed (31 March 2021) | Viewed by 18960

Special Issue Editors

Prof. Dr. Stefano Palmucci

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Guest Editor
Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, Radiology I Unit, University Hospital “Policlinico-Vittorio Emanuele”, University of Catania, Catania, Italy
Interests: chest imaging; HRCT; interstitial lung diseases; quantitative HRCT; functional MRI of the abdomen; liver and pancreatic diseases on imaging
Special Issues, Collections and Topics in MDPI journals
Dr. Gianluca Sambataro

E-Mail Website
Co-Guest Editor
Regional Referral Centre for Rare Lung Diseases, A. O. U. “Policlinico-Vittorio Emanuele” Department of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy
Interests: interstitial pneumonia with autoimmune features; IPAF; interstitial lung diseases associated with systemic autoimmune diseases
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Interstitial lung diseases (ILDs) include different lung conditions, which may be classified into four categories: (1) diseases with known causes (due to inorganic or organic exposure, smoking), (2) idiopathic interstitial pneumonias (IIPs), (3) granulomatous diseases (e.g., sarcoidosis, chronic hypersensitivity pneumonias), and (4) other or miscellaneous disorders (e.g., Langerhans cell histiocytosis, eosinophilic pneumonias, and lymphangioleiomyomatosis).

Among IIPs, idiopathic pulmonary fibrosis (IPF) is characterized by the worst prognosis, showing an overall survival of 3–5 years from the diagnosis. High-resolution computed tomography (HRCT) may provide a diagnosis of disease in cases of typical radiological patterns. In cases of inconclusive radiological appearances, a multidisciplinary assessment is required, involving pulmonologists, radiologists, pathologists, rheumatologists, and surgeons. Some radiological patterns (non-specific interstitial pneumonias, organizing pneumonias) may be secondary to rheumatic diseases such as scleroderma, myositis, or rheumatoid arthritis. Other diseases with progressive fibrotic patterns (e.g., chronic hypersensitivity pneumonia or sarcoidosis) should be differentiated from IPF. A quantitative assessment of many interstitial diseases represents a promising tool for the diagnosis and progression of IIPs, even if related to a rheumatic disorder. The differential diagnosis between these conditions has a pivotal role, considering that several forms can benefit from antifibrotic drugs, whereas other conditions are generally treated with immunosuppressants.

In addition, in the last year the SARS-CoV-2 outbreak caused acute interstitial pneumonias in about half a million people around the world. Our knowledge is currently limited about the risk of acute ILD exacerbation due to a concomitant infection by SARS-CoV-2. Other interesting topics include the possible increased risk of infection in ILD and the risk of chronic ILD secondary to coronavirus infection.

Therefore, this Special Issue would like focus on the main important features of ILDs, emphasizing the playmaker role of HRCT in disease characterization and monitoring, and the diagnostic capability of multidisciplinary discussion in the diagnosis of ILDs. Given the clinical and radiological aims of this Special Issue, studies focusing on the role of complementary exams (e.g., lung quantitative analysis of CT examinations), serological parameters, and clinical features that are potentially useful in the prognostic and diagnostic assessment of conditions underlying ILD are welcome.

Prof. Dr. Stefano Palmucci
Guest Editor
Dr. Gianluca Sambataro
Co-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 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 (6 papers)

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Research

10 pages, 1857 KiB  
Article
Liquid Biopsy Is a Promising Tool for Genetic Testing in Idiopathic Pulmonary Fibrosis
by Pierlorenzo Pallante, Umberto Malapelle, Mariantonia Nacchio, Roberta Sgariglia, Domenico Galati, Ludovica Capitelli, Serena Zanotta, Mario Galgani, Erica Piemonte, Alessandro Sanduzzi Zamparelli, Gaetano Rea and Marialuisa Bocchino
Diagnostics 2021, 11(7), 1202; https://doi.org/10.3390/diagnostics11071202 - 02 Jul 2021
Cited by 6 | Viewed by 2023
Abstract
Liquid biopsy, which allows the isolation of circulating cell-free (ccf) DNA from blood, is an emerging noninvasive tool widely used in oncology for diagnostic and prognosis purposes. Previous data have shown that serum cfDNA discriminates idiopathic pulmonary fibrosis (IPF) from other interstitial lung [...] Read more.
Liquid biopsy, which allows the isolation of circulating cell-free (ccf) DNA from blood, is an emerging noninvasive tool widely used in oncology for diagnostic and prognosis purposes. Previous data have shown that serum cfDNA discriminates idiopathic pulmonary fibrosis (IPF) from other interstitial lung diseases. Our study aimed to measure plasma levels of ccfDNA in 59 consecutive therapy-naive and clinically stable IPF patients. The single nucleotide polymorphism (SNP) of the MUC5B gene promoter (rs35705950), associated with increased susceptibility of developing IPF, has been sought in plasma cfDNA and genomic DNA for comparison. Thirty-five age- and sex-matched healthy volunteers were recruited as the control group. Our results show that concentrations of small-size ccfDNA fragments were significantly higher in IPF patients than in controls and inversely correlated with lung function deterioration. Moreover, the median level of 104 ng/mL allowed discriminating patients with mild disease from those more advanced. The rs35705950 polymorphism was found in 11.8% of IPF patients and 8% of controls, with no differences. Complete concordance between ccfDNA and genomic DNA was detected in all control samples, while four out of seven IPF cases (57%) carrying the rs35705950 polymorphism were discordant from genomic DNA (7% of total IPF). Liquid biopsy is a suitable tool with optimistic expectations of application in the field of IPF. In analogy with cancer biology, finding some discrepancies between ccfDNA and genomic DNA in IPF patients suggests that the former may convey specific genetic information present in the primary site of the disease. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases 2.0)
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12 pages, 3428 KiB  
Article
Evaluation of Correlations between Genetic Variants and High-Resolution Computed Tomography Patterns in Idiopathic Pulmonary Fibrosis
by Elisa Baratella, Barbara Ruaro, Fabiola Giudici, Barbara Wade, Mario Santagiuliana, Francesco Salton, Paola Confalonieri, Michele Simbolo, Aldo Scarpa, Saverio Tollot, Cristina Marrocchio, Maria Assunta Cova and Marco Confalonieri
Diagnostics 2021, 11(5), 762; https://doi.org/10.3390/diagnostics11050762 - 23 Apr 2021
Cited by 47 | Viewed by 2599
Abstract
Background. Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease (ILD). This prospective observational study aimed at the evaluation of any correlation between genetic variants associated with IPF susceptibility and high-resolution computed tomography (HRCT) patterns. It also aimed at evidencing any [...] Read more.
Background. Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease (ILD). This prospective observational study aimed at the evaluation of any correlation between genetic variants associated with IPF susceptibility and high-resolution computed tomography (HRCT) patterns. It also aimed at evidencing any differences in the HRTC pattern between the familial and sporadic form at diagnosis and after two years. Methods. A total of 65 IPF patients (mean age at diagnosis 65 ± 10) were enrolled after having given written informed consent. HRCT and genetic evaluations were performed. Results. A total of 19 familial (mean age 62 ± 15) and 46 sporadic (mean age 70 ± 9) IPF patients were enrolled. A statistically significant difference was evidenced in the HRTC pattern at diagnosis between the two groups. Sporadic IPF patients had a predominantly usual interstitial pneumonia (UIP) pattern compared with those patients with familial IPF (60.0% vs. 21.1%, respectively). Moreover, familial IPF patients had more alternative diagnoses than those with sporadic IPF (31.6% vs. 2.2%, respectively). Furthermore, there was a slight increase in the typical UIP pattern in the familial IPF group at two years from diagnosis. Conclusions. Genetic factors play a pivotal role in the risk of developing IPF. However, further studies are required to clarify how these genetic factors may guide clinical treatment decisions. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases 2.0)
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14 pages, 1690 KiB  
Article
Interstitial Score and Concentrations of IL-4Rα, PAR-2, and MMP-7 in Bronchoalveolar Lavage Fluid Could Be Useful Markers for Distinguishing Idiopathic Interstitial Pneumonias
by Magdalena Bruzova, Martina Pavlova, Radoslav Matej, Martina Sterclova and Martina Vasakova
Diagnostics 2021, 11(4), 693; https://doi.org/10.3390/diagnostics11040693 - 13 Apr 2021
Cited by 6 | Viewed by 1890
Abstract
Idiopathic interstitial pneumonia (IIP) entails a variable group of lung diseases of unknown etiology. Idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, interstitial lung diseases related to connective tissue disease (CTD-ILD), and hypersensitivity pneumonitis (HP) can manifest with similar clinical, radiological, and histopathological features. In [...] Read more.
Idiopathic interstitial pneumonia (IIP) entails a variable group of lung diseases of unknown etiology. Idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, interstitial lung diseases related to connective tissue disease (CTD-ILD), and hypersensitivity pneumonitis (HP) can manifest with similar clinical, radiological, and histopathological features. In a differential diagnosis, biomarkers can play a significant role. We assume that levels of specific cyto- or chemokines or their receptors can signal pathogenetic processes in the lungs. Eighty patients with different types of idiopathic interstitial pneumonia were enrolled in this study. Cell counts and concentrations of tumor necrosis factor (TNF)-α, interleukin-4 receptor α, proteinase-activated receptor (PAR)-2, matrix metalloproteinase (MMP)-7, and B cell-activating factor were measured in bronchoalveolar lavage fluid using commercial ELISA kits. High resolution computer tomography results were evaluated using alveolar and interstitial (IS) score scales. Levels of TNF-α were significantly higher in HP compared to fibrosing IIP (p < 0.0001) and CTD-ILD (p = 0.0381). Concentrations of IL-4Rα, PAR-2, and MMP-7 were positively correlated with IS (p = 0.0009; p = 0.0256; p = 0.0015, respectively). Since TNF-α plays a major role in inflammation, our results suggest that HP is predominantly an inflammatory disease. From the positive correlation with IS we believe that IL-4Rα, PAR-2, and MMP-7 could serve as fibroproliferative biomarkers in differential diagnosis of IIP. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases 2.0)
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11 pages, 4504 KiB  
Article
Quantitative Evaluation of Fibrosis in IPF Patients: Meaning of Diffuse Pulmonary Ossification
by Monica Palermo, Francesco Tiralongo, Giulio Distefano, Ada Vancheri, Mauro Giuffrè, Fabio Pino, Pietro Valerio Foti, Gianluca Sambataro, Carlo Vancheri, Stefano Palmucci and Antonio Basile
Diagnostics 2021, 11(1), 113; https://doi.org/10.3390/diagnostics11010113 - 12 Jan 2021
Cited by 4 | Viewed by 2634
Abstract
To investigate the role of diffuse pulmonary ossification (DPO) in disease severity in a population of Idiopathic Pulmonary Fibrosis (IPF) patients. This retrospective study was carried out on 95 IPF patients—44 with DPO on high resolution computed tomography (HRCT) and 51 with no [...] Read more.
To investigate the role of diffuse pulmonary ossification (DPO) in disease severity in a population of Idiopathic Pulmonary Fibrosis (IPF) patients. This retrospective study was carried out on 95 IPF patients—44 with DPO on high resolution computed tomography (HRCT) and 51 with no calcifications detected on HRCT. Pulmonary Function Tests (PFTs) acquired nearest to the HRCT were collected. Images were analyzed by two radiologists using a qualitative method, based on HRCT fibrosis visual score, and using a quantitative method, based on histogram-based analysis. The Spearman’s rank correlation coefficient was used to measure the strength and direction of the linear relationship between HRCT fibrosis score and PFTs; in addition, Spearman’s rank correlation coefficient was used to explore the relationships between HRCT fibrosis score and quantitative index and between quantitative indexes and PFTs. A weak correlation between HRCT fibrosis score and PFTs was proven (r =–0.014 and p = 0.9347 for FVC (Forced Vital Capacity), r = −0.379 and p = 0.0174 for DLCO (Carbon monoxide diffusing capacity)). We found a moderate negative correlation between HRCT fibrosis score and kurtosis (r = −0.448, p = 0.004272) and skewness (r = −0.463, p = 0.003019) and a weak positive correlation with High Attenuation Area (HAA)% (r = 0.362, p = 0.0235). Moreover, a moderate linear correlation between Quantitative Indexes and FVC (r = 0.577, p = 0.000051 for kurtosis and FVC, r = 0.598, p = 0.000023 for skewness and FVC, r = −0.519, p = 0.0000364 for HAA% and FVC) and between quantitative indexes and DLCO (r = 0.469, p = 0.001508 for kurtosis, and DLCO, r = 0.474, p = 0.001309 for skewness and DLCO, r = −0.412, p = 0.005996 for HAA% and DLCO) was revealed. To better investigate the influence of DPO in disease progression, a longitudinal evaluation should be performed. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases 2.0)
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11 pages, 1006 KiB  
Article
Multidisciplinary Evaluation of Interstitial Lung Diseases: New Opportunities Linked to Rheumatologist Involvement
by Enrico De Lorenzis, Silvia Laura Bosello, Francesco Varone, Giacomo Sgalla, Lucio Calandriello, Gerlando Natalello, Bruno Iovene, Giuseppe Cicchetti, Laura Gigante, Lucrezia Verardi, Elisa Gremese, Luca Richeldi and Anna Rita Larici
Diagnostics 2020, 10(9), 664; https://doi.org/10.3390/diagnostics10090664 - 02 Sep 2020
Cited by 15 | Viewed by 3002
Abstract
Multidisciplinary team (MDT) discussion is the gold standard in the management of interstitial lung disease (ILD). The rheumatologist is not routinely involved in MDT, even if up to 20% of ILD are related to systemic autoimmune rheumatic diseases (SARD). The study aims to [...] Read more.
Multidisciplinary team (MDT) discussion is the gold standard in the management of interstitial lung disease (ILD). The rheumatologist is not routinely involved in MDT, even if up to 20% of ILD are related to systemic autoimmune rheumatic diseases (SARD). The study aims to assess the agreement and its variation over time between rheumatologists and pulmonologists in the screening of SARD and between rheumatologists and an MDT extended to rheumatologists (eMDT) in evaluating the progression of SARD. We computed the agreement between the pulmonologist and rheumatologist in the identification of red flags for SARDs of 81 ILD cases and between the rheumatologist alone and eMDT in the confirmation of 70 suspected SARD-ILD progressions. The agreement between rheumatologists and pulmonologists was moderate for the detection of autoimmunity test positivity (κ = 0.475, p < 0.001) and family history of SARD (κ = 0.491, p < 0.001) and fair for the identification of extrapulmonary symptoms (κ = 0.225, p = 0.064) or routine laboratory abnormalities consistent with SARD. The average agreement between the rheumatologist and eMDT in the identification of ILD progression was moderate (κ = 0.436, p < 0.001). The class of agreement improved from the first to the third semester. The average agreement with the rheumatologist ranged from fair to moderate, suggesting that a shared evaluation of SARD-ILD in eMDT could improve the diagnostic work-up and the evaluation of ILD progression. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases 2.0)
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14 pages, 1399 KiB  
Article
The Model for Early COvid-19 Recognition (MECOR) Score: A Proof-of-Concept for a Simple and Low-Cost Tool to Recognize a Possible Viral Etiology in Community-Acquired Pneumonia Patients during COVID-19 Outbreak
by Gianluca Sambataro, Mauro Giuffrè, Domenico Sambataro, Andrea Palermo, Giovanna Vignigni, Roberto Cesareo, Nunzio Crimi, Sebastiano Emanuele Torrisi, Carlo Vancheri, Lorenzo Malatino, Michele Colaci, Nicoletta Del Papa, Francesca Pignataro, Erik Roman-Pognuz, Massimiliano Fabbiani, Francesca Montagnani, Chiara Cassol, Lorenzo Cavagna, Valentina Zuccaro, Verena Zerbato, Cristina Maurel, Roberto Luzzati and Stefano Di Bellaadd Show full author list remove Hide full author list
Diagnostics 2020, 10(9), 619; https://doi.org/10.3390/diagnostics10090619 - 21 Aug 2020
Cited by 31 | Viewed by 5843
Abstract
This study aims to assess the peripheral blood cell count “signature” of Severe Acute Respiratory Syndrome-Coronavirus 2 (SARS-CoV-2) to discriminate promptly between COronaVIrus Disease 19 (COVID-19) and community-acquired pneumonia (CAP). We designed a retrospective case-control study, enrolling 525 patients (283 COVID-19 and 242 [...] Read more.
This study aims to assess the peripheral blood cell count “signature” of Severe Acute Respiratory Syndrome-Coronavirus 2 (SARS-CoV-2) to discriminate promptly between COronaVIrus Disease 19 (COVID-19) and community-acquired pneumonia (CAP). We designed a retrospective case-control study, enrolling 525 patients (283 COVID-19 and 242 with CAP). All patients had a fever and at least one of the following signs: cough, chest pain, or dyspnea. We excluded patients treated with immunosuppressants, steroids, or affected by diseases known to modify blood cell count. COVID-19 patients showed a significant reduction in white blood cells (neutrophils, lymphocytes, monocytes, eosinophils) and platelets. We studied these parameters univariately, combined the significant ones in a multivariate model (AUROC 0.86, Nagelkerke PSEUDO-R2 0.5, Hosmer–Lemeshow p-value 0.9) and examined its discriminative performance in an internally-randomized validation cohort (AUROC 0.84). The cut-off selected according to Youden’s Index (−0.13) showed a sensitivity of 84% and a specificity of 72% in the training cohort, and a sensitivity of 88% and a specificity of 73% in the validation cohort. In addition, we determined the probability of having COVID-19 pneumonia for each Model for possible Early COvid-19 Recognition (MECOR) Score value. In conclusion, our model could provide a simple, rapid, and cheap tool for prompt COVID-19 diagnostic triage in patients with CAP. The actual effectiveness should be evaluated in further, prospective studies also involving COVID-19 patients with negative nasopharyngeal swabs. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases 2.0)
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