Lung Ultrasound B-Lines in the Evaluation of the Extent of Interstitial Lung Disease in Systemic Sclerosis

Bruni, Cosimo; Mattolini, Lavinia; Tofani, Lorenzo; Gargani, Luna; Landini, Nicholas; Roma, Nicola; Lepri, Gemma; Orlandi, Martina; Guiducci, Serena; Bellando-Randone, Silvia; Romei, Chiara; Wang, Yukai; Matucci‐Cerinic, Marco

doi:10.3390/diagnostics12071696

Cited by 12 publications

(22 citation statements)

References 29 publications

(47 reference statements)

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“…The technique offers relatively easy access to the costal and diaphragmatic pleura, being also capable of detecting small volumes of pleural effusion. Moreover, despite its use is limited to the lung surface, interest in using LUS to assess diffuse lung disease is increasing, for example, in adult respiratory distress syndrome in intensive care units, Covid-19 pneumonia and interstitial lung diseases (101)(102)(103)(104)(105). Unfortunately, limited data are available about the use of LUS to monitor lung status in PCF or evaluate RTE.…”

Section: Lung Ultrasoundmentioning

confidence: 99%

Management of respiratory tract exacerbations in people with cystic fibrosis: Focus on imaging

Landini

Ciet²,

Janssens³

et al. 2023

Front. Pediatr.

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Respiratory tract exacerbations play a crucial role in progressive lung damage of people with cystic fibrosis, representing a major determinant in the loss of functional lung tissue, quality of life and patient survival. Detection and monitoring of respiratory tract exacerbations are challenging for clinicians, since under- and over-treatment convey several risks for the patient. Although various diagnostic and monitoring tools are available, their implementation is hampered by the current definition of respiratory tract exacerbation, which lacks objective “cut-offs” for clinical and lung function parameters. In particular, the latter shows a large variability, making the current 10% change in spirometry outcomes an unreliable threshold to detect exacerbation. Moreover, spirometry cannot be reliably performed in preschool children and new emerging tools, such as the forced oscillation technique, are still complementary and need more validation. Therefore, lung imaging is a key in providing respiratory tract exacerbation-related structural and functional information. However, imaging encompasses several diagnostic options, each with different advantages and limitations; for instance, conventional chest radiography, the most used radiological technique, may lack sensitivity and specificity in respiratory tract exacerbations diagnosis. Other methods, including computed tomography, positron emission tomography and magnetic resonance imaging, are limited by either radiation safety issues or the need for anesthesia in uncooperative patients. Finally, lung ultrasound has been proposed as a safe bedside option but it is highly operator-dependent and there is no strong evidence of its possible use during respiratory tract exacerbation. This review summarizes the clinical challenges of respiratory tract exacerbations in patients with cystic fibrosis with a special focus on imaging. Firstly, the definition of respiratory tract exacerbation is examined, while diagnostic and monitoring tools are briefly described to set the scene. This is followed by advantages and disadvantages of each imaging technique, concluding with a diagnostic imaging algorithm for disease monitoring during respiratory tract exacerbation in the cystic fibrosis patient.

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Section: Lung Ultrasoundmentioning

confidence: 99%

Management of respiratory tract exacerbations in people with cystic fibrosis: Focus on imaging

Landini

Ciet²,

Janssens³

et al. 2023

Front. Pediatr.

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“…Lung endothelial damage is central to the pathogenesis of ILD. Histologically, SSc-ILD presents as a picture of nonspecific interstitial pneumonia, unlike idiopathic pulmonary fibrosis, which is usually characterized by the usual picture of interstitial pneumonia [93][94][95][96][97][98]. SSc-ILD is characterized by inflammation early in the disease, extensive endothelial dysfunction, and increased deposition of ECM, particularly collagen produced by activated myofibroblasts in resident tissues [79][80][81][82][83][84][85][86][87].…”

Section: Biomarkers In Systemic Sclerosis Interstitial Lung Diseasementioning

confidence: 99%

“…Approximately 10% of SSc patients are ANA-negative, but both ANA-negative and ANA-positive patients may present with novel antibodies, including anti-elF2B, an-ti-RuvBL1/2 complex, anti-U11/12 RNP, anti-U3RNP, anti-BICD2, anti-Ku and an-ti-PM/Scl [7][8][9][10][84][85][86][87][88][89][90][91]. It is unusual to find two different SSc-specific autoantibodies simultaneously in the same individual [29][30][31][32][33][48][49][50][51][52][53][54][55][92][93][94][95][96][97][98][99]. Mortality in SSc patients is significantly increased and usually related to the life-threatening manifestations of SSc, including PAH, ILD, SRC, cardiac involvement, tumors, and infections, which are also related to immunosuppressive drugs [8,9,34,48,[56][57][58][100][101][102][103][104][105]<...…”

Section: Introductionmentioning

confidence: 99%

Biomarkers in Systemic Sclerosis: An Overview

Di Maggio,

Confalonieri,

Salton

et al. 2023

CIMB

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Systemic sclerosis (SSc) is a complex autoimmune disease characterized by significant fibrosis of the skin and internal organs, with the main involvement of the lungs, kidneys, heart, esophagus, and intestines. SSc is also characterized by macro- and microvascular damage with reduced peripheral blood perfusion. Several studies have reported more than 240 pathways and numerous dysregulation proteins, giving insight into how the field of biomarkers in SSc is still extremely complex and evolving. Antinuclear antibodies (ANA) are present in more than 90% of SSc patients, and anti-centromere and anti-topoisomerase I antibodies are considered classic biomarkers with precise clinical features. Recent studies have reported that trans-forming growth factor β (TGF-β) plays a central role in the fibrotic process. In addition, interferon regulatory factor 5 (IRF5), interleukin receptor-associated kinase-1 (IRAK-1), connective tissue growth factor (CTGF), transducer and activator of transcription signal 4 (STAT4), pyrin-containing domain 1 (NLRP1), as well as genetic factors, including DRB1 alleles, are implicated in SSc damage. Several interleukins (e.g., IL-1, IL-6, IL-10, IL-17, IL-22, and IL-35) and chemokines (e.g., CCL 2, 5, 23, and CXC 9, 10, 16) are elevated in SSc. While adiponectin and maresin 1 are reduced in patients with SSc, biomarkers are important in research but will be increasingly so in the diagnosis and therapeutic approach to SSc. This review aims to present and highlight the various biomarker molecules, pathways, and receptors involved in the pathology of SSc.

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“…The need for a quick and easy method of screening has arisen, as there is a high prevalence of lung involvement in early SSc patients, with a strong impact on prognosis—the presence of ILD has a 2.9-fold higher mortality risk [ 7 ]. Subsequently, lung ultrasound (LUS) has become a widely explored method given its radiation-free nature and possible bedside application [ 8 ]. LUS was proposed for assessment of SSc-ILD, even in its early stages, as it was shown to have a high negative predictive value, according to some studies [ 7 ].…”

Section: Introductionmentioning

confidence: 99%

“…The most-analyzed LUS features for ILD assessment are B-lines, although some have suggested B-lines to be too unreliable and subjective. B-lines are defined as vertical and hyperechogenic lines that arise from the pleural line [ 8 ].…”

Section: Introductionmentioning

confidence: 99%

Pulmonary Ultrasonography in Systemic Sclerosis-Induced Interstitial Lung Disease—A Systematic Review and Meta-Analysis

et al. 2023

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Background: The aim of the current systematic review was to summarize and evaluate the overall advantages of lung ultrasonography (LUS) examination using high-resolution computed tomography (HRCT) as a reference standard in assessing the presence of interstitial lung disease (ILD) in systemic sclerosis (SSc) patients. Methods: Databases PubMed, Scopus, and Web of Science were searched for studies evaluating LUSs in ILD assessments including SSc patients on 1 February 2023. In assessing risk of bias and applicability, the Revised Tool for the Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) was used. A meta-analysis was performed and the mean specificity, sensitivity, and diagnostic odds ratio (DOR) with a 95% confidence interval (CI) were obtained. In addition, in a bivariate meta-analysis, the summary receiver operating characteristic (SROC) curve area was additionally calculated. Results: Nine studies with a total of 888 participants entered the meta-analysis. A meta-analysis was also performed without one study that used pleural irregularity to assess the diagnostic accuracy of LUSs using B-lines (with a total of 868 participants). Overall sensitivity and specificity did not differ significantly, with only the analysis of the B-lines having a specificity of 0.61 (95% CI 0.44–0.85) and a sensitivity of 0.93 (95% CI 0.89–0.98). The diagnostic odds ratio of univariate analysis of the eight studies using the B-lines as a criterion for ILD diagnosis was 45.32 (95% CI 17.88–114.89). The AUC value of the SROC curve was 0.912 (and 0.917 in consideration of all nine studies), which indicates high sensitivity and a low false-positive rate for the majority of the included studies. Conclusions: LUS examination proved to be a valuable tool in discerning which SSc patients should receive additional HRCT scans to detect ILD and therefore reduces the doses of ionizing radiation exposure in SSc patients. However, further studies are needed to achieve consensus in scoring and the evaluation methodology of LUS examination.

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Lung Ultrasound B-Lines in the Evaluation of the Extent of Interstitial Lung Disease in Systemic Sclerosis

Cited by 12 publications

References 29 publications

Management of respiratory tract exacerbations in people with cystic fibrosis: Focus on imaging

Management of respiratory tract exacerbations in people with cystic fibrosis: Focus on imaging

Biomarkers in Systemic Sclerosis: An Overview

Pulmonary Ultrasonography in Systemic Sclerosis-Induced Interstitial Lung Disease—A Systematic Review and Meta-Analysis

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