Lung Ultrasound and High Resolution Computed Tomography in Interstitial Lung Disease Identification in Rheumatoid Patients

Background: Lung ultrasound (LUS) used to identify interstitial syndrome (IS) and pleural thickening related to diffuse parenchymal lung disease (DPLD) has shown significant correlations with ground glass opacity (GGO) on high-resolution computed tomography (HRCT). However, the applicability of LUS in patients with DPLD subtypes as rare cystic lung diseases has not previously been investigated. This study aimed to observe if distinctive LUS findings could be found in patients with lymphangioleiomyomatosis (LAM), pulmonary Langerhans cell histiocytosis (PLCH), and Birt-Hogg-Dubé syndrome (BHDS). Methods: This single centre case-based cross-sectional study of patients diagnosed with LAM, PCLH and BHDS was conducted at a Danish DPLD specialist centre. Patients underwent clinical examination including LUS. LUS findings were compared to findings scored according to a modified Belmaati score on HRCT and reviewed in consensus between two pulmonologists and one radiologist. Results: Twelve patients with HRCT proven cystic lung disease were included, six with LAM, three with PLCH, two with BHDS, and one with uncharacteristic cystic lung disease. The mean age was 48.7 years (SD ± 15.8). In general all had normal LUS findings. IS could not be found in any patients despite GGO presentation on HRCT among 75% of the patients with a Belmaati in the highest category of 0.76–1.00. Pleural thickening on LUS was present in three patients, but with inconsistent findings. Conclusion: This study indicates that LUS has limited value as a diagnostic tool in patients with LAM, PLCH, and BHDS as normal LUS findings did not rule out severe cystic lung disease.

Background:In recent years, a growing interest has grown around interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA). While high resolution computed tomography (HRCT) of the chest remains the diagnostic method of choice, increasing attention has been directed towards lung ultrasound (LUS) in the diagnosis of ILD in connective tissue diseases. LUS allows the detection of artifacts (B-lines) characteristic of ILD, without the need to use ionizing radiation. However, it is not yet well defined how to interpret the LUS findings under suspicion of RA-ILD.Objectives:To determine the cut-off number of LUS B-lines that identifies a significant RA-ILD.Methods:A cross sectional study was conducted on consecutive RA patients with suspected RA-ILD. The inclusion criteria were clinical (dyspnea, velcro sounds), instrumental (suggestive anomalies on conventional radiography, DLco reduction), or in presence of at least two of the following risk factors for RA-ILD: smoking habit, male sex, advanced age, and ACPA presence.Patients underwent LUS, chest HRCT, pulmonary function tests, and clinical evaluation. The diagnosis of RA-ILD was based on a semi-quantitative evaluation of HRCT using a computer-aided method (CaM). The 10% of fibrosis, measured with this method, was considered as a cut-off for the presence of significant RA-ILD. The LUS was carried out in 14 defined intercostal spaces using a linear multifrequency probe 6-18 MHz (MyLab Class C, Esaote S.p.A., Genoa, Italy) and the number of B-lines present in each intercostal space was counted and summed up (Figure 1). The discriminating validity of the LUS versus HRCT has been studied by using the receiver operating characteristic (ROC) curve analysis.Figure 1.LUS B-line illustrative.Results:72 consecutive RA patients (21 male, 51 female) were evaluated, with a mean age of 63.0 (SD 11.5 years), a mean ACPA titre of 327.6 (SD 633.3) U/ml and a rheumatoid factor of 324.6 (SD 748.7) U/ml. The mean estimate of pulmonary fibrosis using the CaM was 11.2% (SD 7.5) at HRCT, while at LUS the mean number of B-lines was 10.6 (SD 15.1). Pulmonary fibrosis of 10% as measured by the CaM at HRCT was detected in 25 patients (34.72%). Applying this HRCT cut-off point as an estimate of significant fibrosis, the presence of 9 B-lines was found to be the optimal cut-off at ROC curve analysis. This LUS cut-off defines the presence of significant fibrosis with a sensitivity of 70.0%, a specificity of 97.62%, and a positive likelihood ratio of 29.4 (Figure 2).Figure 2.Area under the ROC curve to determine the number of B-lines at LUS to define a significant RA-ILD, applying the 10% of fibrosis at chest HRCT measured by OsiriX as external criterion.Conclusion:The present study provided data to determine the number of B-lines to identify a significant RA-ILD. LUS may represent a useful technique to select RA patients to be assessed by chest HRCT.

Introduction: Diagnosis and monitoring of interstitial lung disease (ILD) in children are mainly based on chest computed tomography (CT), resulting in significant exposure to x-rays. In adult patients, studies have shown a good sensitivity of the lung ultrasound (LUS) for detecting interstitial lesions, with a high correlation between LUS and CT images. The aim of this pilot study was to assess in children with ILD: (i) the feasibility of lung ultrasound and (ii) the comparability of LUS and CT images. Methods: LUS was performed in 5 children followed in the RespiRare® centre for an ILD of various aetiologies. Images visualized by LUS were described and compared to those seen at the latest chest CT. Results: The 5 children were 1.5, 2.5, 7, 11 and 16 years old respectively. Interstitial lesions were visualized by LUS in 4 of the 5 children, characterized by the presence of more than 3 confluent or spaced B lines per field, associated with an irregularity of the pleural line. Two children presented sub-pleural consolidations that were also seen on their CT. Bilateral pleural effusion was observed by LUS in one child. Only one child had a normal LUS while his CT showed some scattered micro-nodular lesions. Conclusion: This pilot study demonstrated a good correlation between LUS and CT images. As in adult patients, LUS could be an interesting tool for screening and monitoring children with ILD, reducing their exposure to x-rays in comparison to CT.

Objective To assess the clinical value of lung ultrasound (LUS) B-lines in diagnosis of rheumatoid arthritis (RA) associated interstitial lung diseases (RA-ILD). Methods Forty-five consecutive patients with RA who underwent a high resolution computed tomography (HRCT) scan of the chest, were also examined by LUS for detection of B-lines (within 1 month independently in all patients). The B-lines score was obtained by summing the number of total 50 inter-costal spaces (ICSs) of chest wall. Pulmonary fibrosis was quantified by HRCT as previously described by the 30-point Warrick score. Results B-lines score significantly correlated with the Warrick score [(r=0.778, 95%CI(0.627, 0.872), P 15). Conclusion The data confirm that LUS is a useful technique to identify ILD in RA. In RA-ILD, B-lines correlate significantly with HRCT and are able to identify mild and severe degree of fibrosis. LUS is a promising non-invasive and non-ionizing strategy for screening RA-ILD. Key words: Arthritis, rheumatoid; Interstitial lung disease; Lung ultrasound; B-lines; High resolution CT

Objective To evaluate the application value of cardiopulmonary ultrasound(CPUS) in patients with interstitial lung diseases(ILD). Methods Ninety patients with clinically diagnosed interstitial lung diseases and 30 healthy subjects were included in this study. The conventional echocardiographic parameters and right ventricular free wall longitudinal strain (RVLS_FW) were measured in all patients and healthy subjects. The degree of pulmonary fibrosis or the disease severity of each ILD patient was scored using a semi-quantitative scoring method. Patients′ pulmonary function parameters and clinical outcome score (GAP score) were recorded. Fourteen patients belong to other types of ILD and 19 patients with incomplete clinical data were unable to perform GAP scoring, finally 57 patients′ clinical and ultrasonographic parameters were measured. On the basis of the pulmonary arterial systolic pressure (PASP) obtained by Doppler echocardiography, 90 patients were divided into two groups: ILD patients with pulmonary hypertension(ILDPH, PASP ≥36 mmHg, 1 mmHg=0.133 kPa) and ILD patients without PH (ILDNPH, PASP<36 mmHg). Comparsion between ILDPH, ILDNPH and healthy subjects were then evaluated. According to the GAP score, all cases were divided into three groups: low risk, moderate risk and high risk. Statistical analysis was performed to examine the relationship between CPUS parameters and clinical parameters. Results ①Compared with ILDNPH patients, ILDPH patients had significantly lower right ventricular function, wider basal right ventricular diameter (RVD), proximal right ventricular outflow diameter (DRVOT1) and right pulmonary artery diameter (DRPA). Compared with healthy subjects, ILDNPH patients had mildly lower right ventricular function and wider left pulmonary artery diameter (DLPA ) and right ventricular outflow tract dimensions at the distal or pulmonic valve (DRVOT2). ②A good correlation was found between lung ultrasound (LUS) scores and pulmonary function parameters, especially diffusion capacity for carbon monoxide of the lung(DLco)(r=-0.68, P<0.001). ③With the increased risk of patients, the right ventricular function was gradually decreased, DRVOT2, DRPA, DLPA were gradually widened, and the lung ultrasound score was gradually increased. A weak correlation was found between GAP scores with RVLS_FW, LUS scores, DRPA(r=0.44, 0.34, 0.31, P<0.05). Conclusions CPUS parameters are useful in the assessment of interstitial lung disease and could reflect the prognosis of ILD patients to some extent. Therefore, on the basis of GAP scores, addition of RVLS_FW and LUS score will be more helpful to evaluate the prognosis of ILD patients. Key words: Cardiopulmonary ultrasound; Lung diseases, interstitial; GAP score; Pulmonary function test

Background: Current data have shown that lung ultrasound (LUS) may be useful in the detection of interstitial lung disease (ILD) by the evaluation of B-lines, the sonographic marker of pulmonary interstitial syndrome. Nevertheless, no prospective study has compared LUS to chest X-ray (CXR) for ILD assessment, and there is no general agreement on the specific echographic diagnostic criteria for defining ILD. Objectives: The aims of this study were (1) to compare the accuracy of LUS and CXR in the detection of ILD using high-resolution CT (HRCT) as the gold standard and (2) to compare the accuracy of different echographic diagnostic criteria for ILD diagnosis. Methods: LUS was performed on 104 patients undergoing HRCT for suspected ILD. In 49 patients, a CXR scan performed within 3 months of HRCT was analyzed. ILD was defined as the presence of ≥5 B-lines in ≥3 chest areas. A total B-line score (TBLS) was also calculated, as in previous studies. The observers evaluating LUS and CXR were blinded to the HRCT results and clinical data. Results: On HRCT, ILD was assessed in 50 patients. CXR was specific (91%; 95% CI 80-100) but not sensitive (48%; 95% CI 28-67). Conversely, LUS showed high sensitivity (92%; 95% CI 84-99) and low specificity (79%; 95% CI 69-90). Using a TBLS, sensitivity did not change, while specificity decreased. Conclusions: LUS could be a sensitive tool for ILD detection. CXR and LUS have different but complementary features, and their combined use could reduce the need for HRCT. The use of different diagnostic criteria for defining ILD does not affect sensitivity but influences specificity.

Funding Acknowledgements Type of funding sources: Public Institution(s). Main funding source(s): This project received a Scholarship for Training and Research from the Association of Ischemic Heart Disease and Cardiological Critical Care of the Spanish Society of Cardiology and an Original Research Projects Grant from the Catalan Society of Cardiology. Background Heart failure (HF) is an important prognostic factor in ST-segment elevation myocardial infarction (STEMI). Although most STEMI patients are Killip-Kimball class I (KK-I) at admission, there is a risk of developing HF during hospitalization and follow-up. Lung ultrasound (LUS) is an easy, fast, and accurate technique that allows pulmonary congestion to be diagnosed even in pre-clinical stages. Several risk scores have been developed to identify low-risk STEMI patients, nonetheless any of these scores include LUS assessment. Purpose We evaluated the prognostic value of subclinical congestion assessed by LUS at hospital admission in patients with KK-I STEMI. Primary endpoint was a composite of readmission for HF or new acute coronary syndrome or death during 30-day follow-up. Methods This was a multicenter study prospectively enrolling 300 patients admitted for KK-I STEMI. LUS was performed during the first 24 hours after revascularization by and independent operator and B-lines were counted off-line differentiating between patients with wet lung (≥ 3 B-lines in at least one lung field) or dry lung. To assess whether LUS could improve the predictive capacity of current risk scales, a model with categorical result of LUS (dry/wet lung) and categorical result of Zwolle score (low/higher risk) was compared to the isolated result of Zwolle score and net reclassification index (NRI) was calculated. Results Wet lung patients had a higher risk of developing the follow-up composite endpoint (hazard ratio [HR] 10.8, 95% CI 2.6-45.2, p=0.001). After adjusting by a propensity score including age, sex, hypertension, chronic kidney disease (glomerular filtration rate &amp;lt;60ml/min/1,73m2), previous atrial fibrillation/flutter, anterior STEMI, and LVEF, a LUS finding of wet lung remained an independent risk factor for the composite endpoint (adjusted HR 5.4, 95% CI 1.0-28.7, p=0.049). LUS was able to predict de composite endpoint with 62.5% sensitivity, 86.8% specificity, 11.1% positive predictive value and 98.9% negative predictive value. LUS improved the predictive capacity of the Zwolle score (net reclassification improvement = 0.99, 95% CI 0.29-1.69, p=0.006) to predict the composite endpoint. CONCLUSIONS Early subclinical pulmonary congestion identified by LUS in KK-I STEMI patients at hospital admission is a strong predictor of adverse outcomes at 30-day follow-up. Patients without subclinical congestion have a very low risk of adverse outcomes and can potentially benefit from an early discharge. LUS improves the predictive ability of the Zwolle risk score, allowing a more accurate identification of low-risk patients.

BackgroundMany systemic sclerosis (SSc) patients develop interstitial lung disease (ILD) during the course of the disease. Promising data have recently shown that lung ultrasound (LUS) is able to detect ILD...

BackgroundA high percentage of patients with systemic sclerosis (SSc) develop interstitial lung disease (ILD) during the course of the disease. Promising data have recently shown that lung ultrasound (LUS) is...

This article refers to ‘Lung ultrasound-guided treatmentin ambulatory patients with heart failure: a random-ized controlled clinical trial (LUS-HF study)’ by M.Rivas-Lasarteet al., published in this issue on pages1605–1613.

Background Lung US surface wave elastography (SWE) can noninvasively quantify lung surface stiffness or fibrosis by evaluating the rate of surface wave propagation. Purpose To assess the utility of lung US SWE for evaluation of interstitial lung disease. Materials and Methods In this prospective study, lung US SWE was used to assess 91 participants (women, 51; men, 40; mean age ± standard deviation [SD], 62.4 years ± 12.9) with interstitial lung disease and 30 healthy subjects (women, 16; men, 14; mean age, 45.4 years ± 14.6) from February 2016 through May 2017. Severity of interstitial lung disease was graded as none (healthy lung [F0]), mild (F1), moderate (F2), or severe (F3) based on pulmonary function tests, high-resolution CT, and clinical assessments. We propagated surface waves on the lung through gentle mechanical excitation of the external chest wall and measured the lung surface wave speed with a US probe. Lung US SWE performance was assessed, and the optimal cutoff wave speed values for fibrosis grades F0 through F3 were determined with receiver operating characteristic (ROC) curve analysis. Results Lung US SWE had a sensitivity of 92% (95% confidence intervals [CI]: 84%, 96%; P < .001) and a specificity of 89% (95% CI: 81%, 94%; P < .001) for differentiating between healthy subjects (F0) and participants with any grade of interstitial lung disease (F1-F3). It had a sensitivity of 50% and a specificity of 81% for differentiating interstitial lung disease grades F0-F2 from F3. The sensitivity was 88% and the specificity was 97% for differentiating between F0 and F1. The highest area under the ROC curve (AUC) values were obtained at 200 Hz and ranged from 0.83 to 0.94 to distinguish between healthy subjects and study participants with any interstitial lung disease. Conclusion Lung US surface wave elastography may be adjunct to high-resolution CT for noninvasive evaluation of interstitial lung disease. © RSNA, 2019 Online supplemental material is available for this article. See also the editorial by Verschakelen in this issue.

Systemic juvenile idiopathic arthritis (JIA) associated with lung disease (JIA-LD) is a potentially life threating complication in children with systemic JIA. Although high-resolution computed tomography (HRCT) is considered the gold standard imaging modality for evaluating interstitial lung disease (ILD), lung ultrasound (US) has shown utility for ILD screening in adults with connective tissue diseases at lower cost and without using ionizing radiation. The goals of this pilot study were to describe lung US features in children with known systemic JIA-LD and to assess the feasibility of lung US in this population. Children age <18 years with systemic JIA-LD and healthy controls were enrolled. Lung US acquisition was performed at 14 lung positions. Demographic, clinical, and HRCT data were collected and reviewed. Feasibility was assessed through patient surveys. Lung US findings were qualitatively and semiquantitatively assessed and compared to HRCT findings. Lung US was performed in 9 children with systemic JIA-LD and 6 healthy controls and took 12 minutes on average to perform. Lung US findings in systemic JIA-LD included focal to diffuse pleural irregularity, granularity, and thickening, with associated scattered or coalesced B-lines, and subpleural consolidations. Lung US findings appeared to correspond to HRCT findings. Lung US in systemic JIA-LD reveals highly conspicuous abnormalities in the pleura and subpleura that appear to correlate with peripheral lung findings on HRCT. Lung US is a feasible imaging tool in children even from an early age. This study suggests a potential role of lung US in systemic JIA-LD screening, diagnosis, and/or prognostication.

Performance of Lung Ultrasound as a Screening Tool for Subclinical Rheumatoid Arthritis-Associated Interstitial Lung Disease: A Multicenter Study.

Introduction Lung diseases (LDs) are the most common causes of life-threatening respiratory failure in babies, while mortality of it gets up to 35%. Chest X-ray is the most valuable imaging modality for the diagnosis of LDs, but recently lung ultrasound (LUS) has been introduced, and several studies have already confirmed LUS to be an accurate and reliable method for the diagnosis of LD in infants. The goal of this study was to evaluate errors of hyper- and hypodiagnostics of LD using LUS in neonates and babies with primary LD and LD secondary to congenital heart disease (CHD). Methods We analyzed the results of 241 ultrasonic examinations (heart and lungs) of 131 neonates and babies aged 1 - 246 days of life. Primary PD (neonatal respiratory distress syndrome - NRDS, bronchopulmonary dysplasia and neonatal pneumonia - NP) had 39 patients, CHD (complete atrioventricular canal ventricular septal defect, atrial septal defect, patent ductus arteriosus, coarctation of the aorta) had 47 patients, diseases without heart and pulmonary involvement (gastrointestinal and urinary tract pathology) had 45 patients. A quarter of patients with CHD also had PDs. LUS was performed according to recommendations of Lichtenstein et al. (2004), Picano et al.(2006). We used standard programs to verify the diagnosis. In order to calculate the errors of hyper- and hypodiagnostics of LD regression modeling and classification trees (Statistica for Windows) were used to process the description of lungs and heart in terms of ultrasound examination together with information regarding gender, mode of delivery, gestational age, body weight and length as well as laboratory and instrumental characteristics. Results Our results confirmed the level of 94–95% sensitivity and specificity of LUS for identification of RDS and NP, which is almost the same as in thoracic X-ray. Regression models identified 76% as maximal explained variance for interstitial syndrome described in terms of LUS. LUS explained 42% of variance of right ventricular dimensions. Error of hyper- and hypodiagnostics of pulmonary hypertension reached 0% and 27% respectively, while interstitial syndrome was more informative than alveolar consolidation (score: 22 versus 62 correspondingly). LUS is not useful for differential diagnosis of primary and secondary LD in neonates (errors of hyper- and hypodiagnostics 16% and 35% correspondingly) Conclusions Our results confirmed usefulness and feasibility of LUS in diagnostics of NP and NRDS. In addition, LUS is accurate enough for identification of pulmonary hypertension in patients with CHD.

Background:Interstitial lung disease (ILD) is one of the most common complications and one of the main causes of morbidity and mortality in Systemic Sclerosis (SSc). High-resolution computed tomography (HRCT) is the gold standard for the diagnosis of ILD and it allows its quantification. Among semi-quantitative methods, Goh et al proposed a semi-quantitative scoring system to visually quantify ILD extent, with categorical cut-off of 20% to distinguish limited and extensive parenchymal involvement with prognostic implications. More recently, the use of radiomics has allowed the objective quantification of ILD through the use of dedicated software, which calculate different parameters of lung density.Given the exposure to ionizing radiation that the procedure entails, other methods of ILD evaluation are being studied, among which lung ultrasound (LUS) identifies the B-lines as a main feature of ILD. So far, different evidences have proposed the use of LUS for the screening of ILD, even in the early phases of the disease and in subclinical lung involvement.Objectives:the aim of this study is to test the role of LUS in quantifying the severity of SSc-ILD, evaluated with both semi-quantitative visual radiological and quantitative radiomic scores.Methods:Adult SSc patients classified according to the ACR/EULAR 2013 criteria patients were assessed with pulmonary function test (PFTs), lung ultrasound and HRCT over 60 days. CT images were analysed qualitatively (by presence/absence of ILD), semi-quantitatively (categorical Goh score &lt;20% vs&gt; 20% of extent and the continuous extent Goh score made from 5 levels’ assessment– 0 to 100%) and quantitatively [with the densitometric radiomic data obtained through the Horos software - Mean lung attenuation (MLA), Standard Deviation (SD), Kurtosis, Skewness and Lung volume (LV)]. LUS was used to quantify the B-lines detected in each patient by scanning a total of 13 intercostal spaces, on both anterior and posterior chest wall.Results:Among 59 SSc patients (81% women, mean age 48±14 years, 45% anti-Scl70 positive), 23 (39%) presented ILD on HRCT, of which 14 limited and 9 extensive. The mean visual semi-quantitative score was 6%, ranging from 0 to 66%. Our data showed a significantly different number of B-Lines in ILD vs non-ILD patients (median 38 vs 9, p &lt;.005), a result which was further confirmed among non-ILD vs ILD&gt; 20% (median 47 vs 9, p=.001) and ILD &lt;20% (median 36 vs 9, p=.001) patients. Conversely, the number of B-lines was not statistically different between patients with ILD &lt;20% and &gt;20% (median 47 vs 36, p=.78). We observed a significant negative correlation between the number of B-lines and FVC (r=-.472, p&lt;.05) TLC (r=-.436, p=.003), DLco (r=-.515, p&lt;.001), DLCO/VA (r=.-306, p=.03). Finally, the number of B-lines showed a statistically significant correlation with the Goh score on 5 levels (r=.437, p=.001), MLA (r=.571, p&lt;.001), kurtosis (r=-.285, p=.028), skewness (r=-.370, p = .004) and LV (r=-.277, p=.033). All data were confirmed analysing anterior and posterior B-Lines separately.Conclusion:Our study confirms that LUS represents a useful tool for the identification of SSc-ILD. In addition, we showed that LUS may be useful also for the quantification of the severity of SSc-ILD, by correlating with PFT parameters, radiomics parameters and visual radiological evaluation. Together with the PFTs, LUS could be used to increase the accuracy of the screening and, potentially, of the follow-up of SSc-ILD patients.Disclosure of Interests:Cosimo Bruni: None declared, Lavinia Mattolini: None declared, Lorenzo Tofani: None declared, Luna Gargani Consultant of: GE Healthcare, Philips Healthcare and Caption Health, Nicholas Landini: None declared, Gemma Lepri: None declared, Martina Orlandi: None declared, Serena Guiducci: None declared, Silvia Bellando Randone: None declared, Marco Matucci-Cerinic: None declared