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

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 May 2020) | Viewed by 84485

Special Issue Editors

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, 95123 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. Sebastiano Emanuele Torrisi

E-Mail Website
Guest Editor
Department of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Diseases, University Hospital “Policlinico”, University of Catania, Catania, Italy
Interests: respiratory diseases; interstitial lung diseases; idiopathic pulmonary fibrosis; interstitial pneumonia with autoimmune features

Special Issue Information

Dear Colleagues,

Interstitial lung diseases (ILD) include different lung conditions, which may be classified into four categories: (1) diseases with known causes, (2) idiopathic interstitial pneumonias (IIPs), (3) granulomatous diseases (e.g., sarcoidosis, chronic hypersensitivity pneumonias), and (4) other or miscellaneous disorders (e.g., Langerhan’s 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 pneumologists, radiologists, 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 secondary rheumatic disorder.

Therefore, this Special Issue would like focus on the main important features of interstitial lung diseases, emphasizing the playmaker role of HRCT in disease characterization and monitoring, and the diagnostic capability of multidisciplinary discussion in the diagnosis of ILDs—clinical features that should be considered in the diagnostic approach of suspected idiopathic inflammatory myositis underlying ILD.

Prof. Dr. Stefano Palmucci
Dr. Sebastiano Emanuele Torrisi
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.

Keywords

  • Interstitial lung diseases
  • Idiopathic pulmonary fibrosis
  • High resolution computed tomography
  • Progressive fibrotic interstitial lung diseases
  • Interstitial pneumonia with autoimmune features
  • NSIP
  • CHP
  • OP
  • Sarcoidosis
  • IPF and comorbidities

Related Special Issue

Published Papers (10 papers)

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Editorial

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3 pages, 174 KiB  
Editorial
Multidisciplinary Approach to Interstitial Lung Diseases: Nothing Is Better than All of Us Together
by Carlo Vancheri and Antonio Basile
Diagnostics 2020, 10(7), 488; https://doi.org/10.3390/diagnostics10070488 - 17 Jul 2020
Cited by 1 | Viewed by 2115
Abstract
Interstitial Lung Diseases (ILDs) are a large family of disorders characterized by inflammation and/or fibrosis of areas of the lung dedicated to gas exchange. In this Special Issue entitled “Clinical and Radiological Features of Interstitial Lung Diseases”, we collected a series of contributions [...] Read more.
Interstitial Lung Diseases (ILDs) are a large family of disorders characterized by inflammation and/or fibrosis of areas of the lung dedicated to gas exchange. In this Special Issue entitled “Clinical and Radiological Features of Interstitial Lung Diseases”, we collected a series of contributions in which a multidisciplinary approach was crucial for the correct diagnostic assessment of ILD. Sharing knowledge between different specialties can significantly improve diagnostic approaches and the management of ILD patients. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases)

Research

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19 pages, 32225 KiB  
Article
Complications in Idiopathic Pulmonary Fibrosis: Focus on Their Clinical and Radiological Features
by Federica Galioto, Stefano Palmucci, Giovanna M. Astuti, Ada Vancheri, Giulio Distefano, Francesco Tiralongo, Alessandro Libra, Giacomo Cusumano, Antonio Basile and Carlo Vancheri
Diagnostics 2020, 10(7), 450; https://doi.org/10.3390/diagnostics10070450 - 03 Jul 2020
Cited by 10 | Viewed by 4740
Abstract
Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease with uncertain origins and pathogenesis; it represents the most common interstitial lung disease (ILD), associated with a pathological pattern of usual interstitial pneumonitis (UIP). This disease has a poor prognosis, having the most lethal [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease with uncertain origins and pathogenesis; it represents the most common interstitial lung disease (ILD), associated with a pathological pattern of usual interstitial pneumonitis (UIP). This disease has a poor prognosis, having the most lethal prognosis among ILDs. In fact, the progressive fibrosis related to IPF could lead to the development of complications, such as acute exacerbation, lung cancer, infections, pneumothorax and pulmonary hypertension. Pneumologists, radiologists and pathologists play a key role in the identification of IPF disease, and in the characterization of its complications—which unfortunately increase disease mortality and reduce overall survival. The early identification of these complications is very important, and requires an integrated approach among specialists, in order to plane the correct treatment. In some cases, the degree of severity of patients having IPF complications may require a personalized approach, based on palliative care services. Therefore, in this paper, we have focused on clinical and radiological features of the complications that occurred in our IPF patients, providing a comprehensive and accurate pictorial essay for clinicians, radiologists and surgeons involved in their management. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases)
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12 pages, 2031 KiB  
Article
Performance of Radiomics Features in the Quantification of Idiopathic Pulmonary Fibrosis from HRCT
by Alessandro Stefano, Mauro Gioè, Giorgio Russo, Stefano Palmucci, Sebastiano Emanuele Torrisi, Samuel Bignardi, Antonio Basile, Albert Comelli, Viviana Benfante, Gianluca Sambataro, Daniele Falsaperla, Alfredo Gaetano Torcitto, Massimo Attanasio, Anthony Yezzi and Carlo Vancheri
Diagnostics 2020, 10(5), 306; https://doi.org/10.3390/diagnostics10050306 - 15 May 2020
Cited by 38 | Viewed by 3428
Abstract
Background: Our study assesses the diagnostic value of different features extracted from high resolution computed tomography (HRCT) images of patients with idiopathic pulmonary fibrosis. These features are investigated over a range of HRCT lung volume measurements (in Hounsfield Units) for which no prior [...] Read more.
Background: Our study assesses the diagnostic value of different features extracted from high resolution computed tomography (HRCT) images of patients with idiopathic pulmonary fibrosis. These features are investigated over a range of HRCT lung volume measurements (in Hounsfield Units) for which no prior study has yet been published. In particular, we provide a comparison of their diagnostic value at different Hounsfield Unit (HU) thresholds, including corresponding pulmonary functional tests. Methods: We consider thirty-two patients retrospectively for whom both HRCT examinations and spirometry tests were available. First, we analyse the HRCT histogram to extract quantitative lung fibrosis features. Next, we evaluate the relationship between pulmonary function and the HRCT features at selected HU thresholds, namely −200 HU, 0 HU, and +200 HU. We model the relationship using a Poisson approximation to identify the measure with the highest log-likelihood. Results: Our Poisson models reveal no difference at the −200 and 0 HU thresholds. However, inferential conclusions change at the +200 HU threshold. Among the HRCT features considered, the percentage of normally attenuated lung at −200 HU shows the most significant diagnostic utility. Conclusions: The percentage of normally attenuated lung can be used together with qualitative HRCT assessment and pulmonary function tests to enhance the idiopathic pulmonary fibrosis (IPF) diagnostic process. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases)
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19 pages, 19571 KiB  
Article
Cryptogenic Organizing Pneumonia: Evolution of Morphological Patterns Assessed by HRCT
by Francesco Tiralongo, Monica Palermo, Giulio Distefano, Ada Vancheri, Gianluca Sambataro, Sebastiano Emanuele Torrisi, Federica Galioto, Agata Ferlito, Giulia Fazio, Pietro Valerio Foti, Letizia Antonella Mauro, Carlo Vancheri, Stefano Palmucci and Antonio Basile
Diagnostics 2020, 10(5), 262; https://doi.org/10.3390/diagnostics10050262 - 29 Apr 2020
Cited by 20 | Viewed by 29965
Abstract
To evaluate the radiological findings in patients with cryptogenic organizing pneumonia (COP) before steroid treatment and their behavior after therapy, we retrospectively evaluated a total of 22 patients with a diagnosis of COP made by bronchoalveolar lavage (BAL), biopsy or clinical/radiological features, and [...] Read more.
To evaluate the radiological findings in patients with cryptogenic organizing pneumonia (COP) before steroid treatment and their behavior after therapy, we retrospectively evaluated a total of 22 patients with a diagnosis of COP made by bronchoalveolar lavage (BAL), biopsy or clinical/radiological features, and the patients were followed between 2014 and 2018 at the hospital; the demographic data, symptoms, radiologic findings, diagnostic methods and treatment plans of patients were collected from patients’ hospital records. At least two CT scans of 22 patients (16 female and six men) were evaluated, the first one before starting steroid therapy and the others after therapy. At baseline CT scans, the most common radiological finding was the presence of consolidations (18/22 patients, 81.8%); ground-glass opacities were also very common (15/25, 68.1%). The other findings were as follows: nodules and masses (5/22, 22.7%), atoll sign (4/22, 18.1%), perilobular pattern (3/22, 13.6%) and parenchymal bands (3/22, 13.6%). Two patients had a significant relapse after reducing/interrupting therapy, while three had a complete resolution and are not currently under therapy (maintenance of clinical remission with no oral corticosteroid (OCS)). In High-resolution computed tomography (HRCT) scans after therapy, consolidations were still observable in seven patients (five in new areas of the lung-migratory infiltrates), while most of them disappeared, leaving a residual area of ground glass opacity in two patients. One patient had a residual of the perilobular pattern, with the disappearing of the other findings (consolidations and ground-glass opacities). Two patients developed a fibrosing pattern despite the therapy (9.5%). Cryptogenic organizing pneumonia tends to respond to oral corticosteroid treatment, but some patients may have a null or partial response. We highlight the behavior of this disease after proper therapy. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases)
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12 pages, 914 KiB  
Article
Nailfold Videocapillaroscopy Is a Useful Tool to Recognize Definite Forms of Systemic Sclerosis and Idiopathic Inflammatory Myositis in Interstitial Lung Disease Patients
by Domenico Sambataro, Gianluca Sambataro, Alessandro Libra, Giovanna Vignigni, Fabio Pino, Evelina Fagone, Mary Fruciano, Elisa Gili, Francesca Pignataro, Nicoletta Del Papa and Carlo Vancheri
Diagnostics 2020, 10(5), 253; https://doi.org/10.3390/diagnostics10050253 - 25 Apr 2020
Cited by 15 | Viewed by 2997
Abstract
Nailfold videocapillaroscopy (NVC) is an easy tool used for the assessment of patients with Raynaud’s phenomenon (RP) as possibly associated with systemic sclerosis (SSc). Recent insights have also highlighted its role in the diagnostic assessment of idiopathic inflammatory myopathies (IIMs). The aim of [...] Read more.
Nailfold videocapillaroscopy (NVC) is an easy tool used for the assessment of patients with Raynaud’s phenomenon (RP) as possibly associated with systemic sclerosis (SSc). Recent insights have also highlighted its role in the diagnostic assessment of idiopathic inflammatory myopathies (IIMs). The aim of this study is to describe the diagnostic role of NVC in a series of 361 consecutive patients with interstitial lung disease (ILD). All the patients were assessed by clinical pulmonary and rheumatic examinations, blood exams, high-resolution computed tomography and NVC. NVC was considered positive only in the presence of avascular areas or giant capillaries, but also, the presence of bushy capillaries (BCs) was recorded. NVC was positive in 17.7% of ILD patients and in 78.1% of ILD patients associated with a diagnosis of connective tissue disease (CTD). In 25% of SSc-ILD patients, NVC proved necessary for a correct diagnosis. The presence of BCs and/or NVC positivity in ILD patients with normal levels of creatine phosphokinase is associated with amyopathic IIM, regardless the presence of RP. In conclusion, NVC is useful for the diagnostic assessment of incomplete forms of CTD and in amyopathic IIMs. NVC should be considered in the diagnostic assessment of ILD patients regardless of the presence of RP. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases)
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11 pages, 433 KiB  
Article
Quantification of Ground Glass Opacities Can Be Useful to Describe Disease Activity in Systemic Sclerosis
by Domenico Sambataro, Gianluca Sambataro, Francesca Pignataro, Wanda Maglione, Lorenzo Malatino, Carlo Vancheri, Michele Colaci and Nicoletta Del Papa
Diagnostics 2020, 10(4), 225; https://doi.org/10.3390/diagnostics10040225 - 16 Apr 2020
Cited by 6 | Viewed by 2437
Abstract
Interstitial lung disease (ILD) is the main cause of death in systemic sclerosis (SSc) patients. Usually, patients have lung involvement characterized by ground glass opacities (GGOs), but honeycombing (HC) is also possible. The Wells score is a semi-quantitative index, which is able to [...] Read more.
Interstitial lung disease (ILD) is the main cause of death in systemic sclerosis (SSc) patients. Usually, patients have lung involvement characterized by ground glass opacities (GGOs), but honeycombing (HC) is also possible. The Wells score is a semi-quantitative index, which is able to assess ILD by distinguishing its main components. The aim of this work is to evaluate the Wells score in relation to the disease activity (DA) index. We enrolled 40 consecutive SSc-ILD patients (26 diffuse cutaneous form, dcSSc, and 14 limited form, lcSSc). All patients were evaluated by the European Scleroderma Study Group (ESSG) index, high-resolution computed tomography, transthoracic echocardiogram, pulmonary function tests (PTSs), and nailfold videocapillaroscopy for the number of microhemorrhages (NEMO) score. In our study, the total extent of ILD (TE-ILD), fibrosis and GGOs correlated with dyspnea (p = 0.03, 0.01 and 0.01 respectively), but not with the ESSG index. Considering only the dcSSc patients, TE-ILD and GGOs correlated with the ESSG index (r = 0.5 p = 0.009), while fibrosis grade correlated with disease duration and systolic pulmonary artery pressure. In conclusion, our data suggest that GGO correlates with DA, while fibrosis may be a sign of disease damage. The quantification of pulmonary involvement using the Wells score can be a useful tool for assessing the appropriate treatment in SSc patients. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases)
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17 pages, 3782 KiB  
Article
Morphological Patterns of Sarcoidosis and Clinical Outcome: Retrospective Analysis through a Multidisciplinary Approach
by Giulio Distefano, Ada Vancheri, Monica Palermo, Francesco Tiralongo, Pietro Valerio Foti, Letizia Antonella Mauro, Carlo Vancheri, Antonio Basile and Stefano Palmucci
Diagnostics 2020, 10(4), 212; https://doi.org/10.3390/diagnostics10040212 - 11 Apr 2020
Cited by 3 | Viewed by 3109
Abstract
The aim of this work was to verify the correlations between different pulmonary morphological patterns and functional outcomes in sarcoidosis patients, using a validated score for the comparison between the high-resolution computed tomography (HRCT) of patients belonging to different imaging patterns. From the [...] Read more.
The aim of this work was to verify the correlations between different pulmonary morphological patterns and functional outcomes in sarcoidosis patients, using a validated score for the comparison between the high-resolution computed tomography (HRCT) of patients belonging to different imaging patterns. From the electronic database of the reference center for interstitial lung diseases of our University Hospital, we retrospectively selected 55 patients with a diagnosis of sarcoidosis according to the American Thoracic Society (ATS) criteria; we evaluated the initial HRCT examination and pulmonary function tests collected at baseline and after a year. Patients were divided into typical (48% of patients) and atypical (52%) HRCT patterns, and a computer tomography activity score (CTAS) was associated with each HRCT appearance detected; clinical history, impact of therapy, and extra-thoracic locations were also considered. We found that worsening of diffusing capacity for carbon monoxide (DLCO) is related to the CTAS (r = −0.20, p = 0.01), and there was an inverse correlation between the variation of forced vital capacity (FVC) and the value of the CTAS (r = −0.30, p = 0.23) in the subgroup of patients with atypical patterns. CTAS were higher in patients with extra-pulmonary localizations (p = 0.05) and the subgroup of patients with extra-thoracic locations and atypical manifestations had a greater worsening in terms of variation of FVC (p = 0.03) and DLCO% (p = 0.04). No difference between treated and untreated patients was found. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases)
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Review

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18 pages, 11369 KiB  
Review
Cystic Interstitial Lung Diseases: A Pictorial Review and a Practical Guide for the Radiologist
by Giulia Aquilina, Daniele Carmelo Caltabiano, Federica Galioto, Giovanna Cancemi, Fabio Pino, Ada Vancheri, Carlo Vancheri, Pietro Valerio Foti, Letizia Antonella Mauro and Antonio Basile
Diagnostics 2020, 10(6), 346; https://doi.org/10.3390/diagnostics10060346 - 27 May 2020
Cited by 5 | Viewed by 12701
Abstract
A cyst is a round circumscribed area of low attenuation, surrounded by epithelial or fibrous wall. Cysts can frequently occur on chest computed tomography (CT) and high-resolution computed tomography (HRCT); multiple parenchymal cysts of the lungs are the most typical feature of cystic [...] Read more.
A cyst is a round circumscribed area of low attenuation, surrounded by epithelial or fibrous wall. Cysts can frequently occur on chest computed tomography (CT) and high-resolution computed tomography (HRCT); multiple parenchymal cysts of the lungs are the most typical feature of cystic lung interstitial diseases, characterizing a wide spectrum of diseases—ranging from isolated lung disorders up to diffuse pulmonary diseases. The aim of this review is to analyze scientific literature about cystic lung interstitial diseases and to provide a practical guide for radiologists, focusing on the main morphological features of pulmonary cysts: size, shape, borders, wall, location, and distribution. These features are shown on free-hand drawings and related to HRCT images, in order to help radiologists pursue the correct differential diagnosis between similar conditions. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases)
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16 pages, 5157 KiB  
Review
HRCT Patterns of Drug-Induced Interstitial Lung Diseases: A Review
by Giulio Distefano, Luigi Fanzone, Monica Palermo, Francesco Tiralongo, Salvatore Cosentino, Corrado Inì, Federica Galioto, Ada Vancheri, Sebastiano E. Torrisi, Letizia A. Mauro, Pietro V. Foti, Carlo Vancheri, Stefano Palmucci and Antonio Basile
Diagnostics 2020, 10(4), 244; https://doi.org/10.3390/diagnostics10040244 - 22 Apr 2020
Cited by 26 | Viewed by 13148
Abstract
Interstitial Lung Diseases (ILDs) represent a heterogeneous group of pathologies, which may be related to different causes. A low percentage of these lung diseases may be secondary to the administration of drugs or substances. Through the PubMed database, an extensive search was performed [...] Read more.
Interstitial Lung Diseases (ILDs) represent a heterogeneous group of pathologies, which may be related to different causes. A low percentage of these lung diseases may be secondary to the administration of drugs or substances. Through the PubMed database, an extensive search was performed in the fields of drug toxicity and interstitial lung disease. We have evaluated the different classes of drugs associated with pulmonary toxicity. Several different high resolution computed tomography (HRCT) patterns related to pulmonary drug toxicity have been reported in literature, and the most frequent ILDs patterns reported include Nonspecific Interstitial Pneumonia (NSIP), Usual Interstitial Pneumonia (UIP), Hypersensitivity Pneumonitis (HP), Organizing Pneumonia (OP), Acute Respiratory Distress Syndrome (ARDS), and Diffuse Alveolar Damage (DAD). Finally, from the electronic database of our Institute we have selected and commented on some cases of drug-induced lung diseases related to the administration of common drugs. As the imaging patterns are rarely specific, an accurate evaluation of the clinical history is required and a multidisciplinary approach—involving pneumologists, cardiologists, radiologists, pathologists, and rheumatologists—is recommended. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases)
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23 pages, 1488 KiB  
Review
Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them?
by Domenico Sambataro, Gianluca Sambataro, Francesca Pignataro, Giovanni Zanframundo, Veronica Codullo, Evelina Fagone, Emanuele Martorana, Francesco Ferro, Martina Orlandi, Nicoletta Del Papa, Lorenzo Cavagna, Lorenzo Malatino, Michele Colaci and Carlo Vancheri
Diagnostics 2020, 10(4), 208; https://doi.org/10.3390/diagnostics10040208 - 09 Apr 2020
Cited by 31 | Viewed by 8670
Abstract
The diagnostic assessment of patients with Interstitial Lung Disease (ILD) can be challenging due to the large number of possible causes. Moreover, the diagnostic approach can be limited by the severity of the disease, which may not allow invasive exams. To overcome this [...] Read more.
The diagnostic assessment of patients with Interstitial Lung Disease (ILD) can be challenging due to the large number of possible causes. Moreover, the diagnostic approach can be limited by the severity of the disease, which may not allow invasive exams. To overcome this issue, the referral centers for ILD organized Multidisciplinary Teams (MDTs), including physicians and experts in complementary discipline, to discuss the management of doubtful cases of ILD. MDT is currently considered the gold standard for ILD diagnosis, but it is not often simple to organize and, furthermore, rheumatologists are still not always included. In fact, even if rheumatologic conditions represent a common cause of ILD, they are sometimes difficult to recognize, considering the variegated clinical features and their association with all possible radiographic patterns of ILD. The first objective of this review is to describe the clinical, laboratory, and instrumental tests that can drive a diagnosis toward a possible rheumatic disease. The secondary objective is to propose a set of first-line tests to perform in all patients in order to recognize any possible rheumatic conditions underlying ILD. Full article
(This article belongs to the Special Issue Clinical and Radiological Features of Interstitial Lung Diseases)
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