Pulmonary High-resolution CT Research Articles

Artificial intelligence-based quantification of pulmonary HRCT (AIqpHRCT) for the evaluation of interstitial lung disease in patients with inflammatory rheumatic diseases

High-resolution computed tomography (HRCT) is important for diagnosing interstitial lung disease (ILD) in inflammatory rheumatic disease (IRD) patients. However, visual ILD assessment via HRCT often has high inter-reader variability. Artificial intelligence (AI)-based techniques for quantitative image analysis promise more accurate diagnostic and prognostic information. This study evaluated the reliability of artificial intelligence-based quantification of pulmonary HRCT (AIqpHRCT) in IRD-ILD patients and verified IRD-ILD quantification using AIqpHRCT in the clinical setting. Reproducibility of AIqpHRCT was verified for each typical HRCT pattern (ground-glass opacity [GGO], non-specific interstitial pneumonia [NSIP], usual interstitial pneumonia [UIP], granuloma). Additional, 50 HRCT datasets from 50 IRD-ILD patients using AIqpHRCT were analysed and correlated with clinical data and pulmonary lung function parameters. AIqpHRCT presented 100% agreement (coefficient of variation = 0.00%, intraclass correlation coefficient = 1.000) regarding the detection of the different HRCT pattern. Furthermore, AIqpHRCT data showed an increase of ILD from 10.7 ± 28.3% (median = 1.3%) in GGO to 18.9 ± 12.4% (median = 18.0%) in UIP pattern. The extent of fibrosis negatively correlated with FVC (ρ=-0.501), TLC (ρ=-0.622), and DLCO (ρ=-0.693) (p < 0.001). GGO measured by AIqpHRCT also significant negatively correlated with DLCO (ρ=-0.699), TLC (ρ=-0.580) and FVC (ρ=-0.423). For the first time, the study demonstrates that AIpqHRCT provides a highly reliable method for quantifying lung parenchymal changes in HRCT images of IRD-ILD patients. Further, the AIqpHRCT method revealed significant correlations between the extent of ILD and lung function parameters. This highlights the potential of AIpqHRCT in enhancing the accuracy of ILD diagnosis and prognosis in clinical settings, ultimately improving patient management and outcomes.

Correlation of diaphragmatic mobility and thickening assessed by lung ultrasound with severity of interstitial lung disease.

Studies conducted in interstitial lung disease (ILD) patients to assess diaphragmatic excursion and thickening fraction suggest a weak to strong correlation with pulmonary function parameters. However, diaphragmatic excursion velocity, a novel imaging marker, has not been correlated with pulmonary function and high-resolution computed tomography (HRCT) fibrosis score in ILD patients previously. We conducted a cross-sectional analytical study in 40 ILD patients during quiet (QB) and deep breathing (DB) to measure diaphragmatic thickening, excursion and excursion velocity using transthoracic ultrasound and correlated them with pulmonary function parameters and HRCT fibrosis score. Most diaphragm parameters in DB correlated more strongly with lung function parameters compared to quiet breathing. Right diaphragmatic excursion, during QB and DB, showed positive correlations with forced vital capacity (FVC) z-score (r = 0.591, 0.676) and diffusion capacity of the lung for carbon monoxide (DLCO) z-score (r = 0.437, 0.438), and negative correlations with HRCT fibrosis score (r = -0.439, -0.425), respectively. In addition, right diaphragmatic velocity exhibited positive correlations with FVC z-score (r = 0.388, 0.667) and DLCOz-score (r = 0.139, 0.412), and negative correlations with HRCT fibrosis score (r = -0.454, -0.445). Right diaphragm thickening fraction showed positive correlations with FVC z-score (r = 0.330, 0.460) and DLCOz-score (r = 0.400, 0.426), and negative correlations with HRCT fibrosis score (r = -0.199, -0.237). Similarly, right diaphragmatic thickness indicated positive correlations with FVC z-score (r = 0.526, 0.614) and DLCOz-score (r = 0.298, 0.298), and negative correlations with HRCT fibrosis score (r = -0.398, -0.401). Diaphragmatic excursion velocity during DB showed a weak to moderate correlation with pulmonary function parameters and HRCT fibrosis score and may be utilized as a surrogate marker in ILD patients unable to perform pulmonary function tests or undergo sequential HRCT thorax in follow-up.

Impact of Bronchoscopic Lung Volume Reduction with Valves on the Pulmonary Gas Exchange.

Introduction: Bronchoscopic lung volume reduction (BLVR) with endobronchial valves has been shown to be a safe and effective treatment for patients with severe lung emphysema. Previous studies have reported a benefit in pulmonary function, exercise capacity, and quality of life after BLVR-treatment. The effect of BLVR with valves on the pulmonary gas exchange and its association with clinical outcomes has not been analyzed to date. The primary goal of this study was to investigate the impact of BLVR on the pulmonary gas exchange and the impact of the target lobe selection in patients with discordant target lobes in high-resolution computed tomography (HRCT) scan and perfusion scan on the pulmonary gas exchange and clinical outcomes. Methods: In this single-center study, we retrospectively analyzed pulmonary function tests, 6-min-walk-tests, HRCT scans, perfusion scans, and blood gas analyses in 77 patients over the course of 6 months following BLVR treatment. Results: We observed that complete lobar occlusion with bronchoscopic valves leads to a transient impairment of pulmonary gas exchange. Despite this, an overall positive clinical outcome could be shown in patients treated with endobronchial valves. If the target lobe selection based on HRCT and perfusion scans is discrepant, a selection based on the HRCT scan tends to be associated with a better outcome than a selection based on the perfusion scan. Conclusions: Complete lobar occlusion with bronchoscopic valves leads to a transient impairment of pulmonary gas exchange but nevertheless results in an overall positive clinical outcome.

Hydrochloric Acid-Induced Acute Lung Injury Models: Dynamic Change and Quantitative Analysis of Modified Lung Ultrasound Scoring System and High-Resolution Computed Tomography

ObjectiveAcute lung injury (ALI) has become a research hotspot due to its significant public health impact. To explore the value of the use of modified lung ultrasound (MLUS) scoring system for evaluating ALI using a rabbit model of ALI induced by hydrochloric acid (HCl) and investigate its correlation with high-resolution computed tomography (HRCT) and histopathological scores. MethodsTwenty New Zealand laboratory rabbits were randomly assigned to control group (N = 5) and 3 experimental groups (N = 5 each). The control group received instillation of physiological saline, while the 3 experimental groups received 2 mL/kg of different doses of HCl instillation (mild group: pH 1.5, moderate group: pH 1.2, and severe group: pH 1.0) through the trachea under ultrasound guidance. Pulmonary ultrasound (using Mindray Reason9 linear array probes with frequency of 6-15 mHz) and HRCT examinations were performed before modeling (0H) and at 1H, 2H, 4H, 8H, 12H after modeling. The experimental rabbits were sacrificed at 12H for examination of gross lung morphology and hematoxylin-eosin-stained histopathological sections. The correlation of MLUS scores with HRCT/histopathological scores was assessed. ResultsAll rabbits in the experimental groups showed oxygenation index PaO₂/FiO₂<300. Successful establishment of ALI model was proven by autopsy (successful modeling rate: 100%). The pathological damage increased with increase in HCl dosage. MLUS scores showed a positive correlation with HRCT scores/pathological severity. There was a strong positive correlation between MLUS scores and histopathological scores (r = 0.963, p < 0.05) as well as between HRCT scores and histopathological scores (r = 0.932, p < 0.05). ConclusionTranstracheal injection of different dosages of HCl under ultrasound guidance induced different degrees of ALI. The MLUS scoring system can be used for semiquantitative evaluation of ALI, and is suitable as a screening tool.

Clinical analysis of 4 children with cryptogenic organizing pneumonia

Objective: To analyze the clinical features of children with cryptogenic organizing pneumonia (COP) confirmed by pathology. Methods: The clinical manifestations, imaging, pathology, treatment and outcome data of 4 children with COP confirmed by thoracoscopic lung biopsy were retrospectively analyzed, who were hospitalized at Respiratory Department of Shenzhen Children's Hospital from January 2004 to December 2022. Results: All of the 4 patients were male, the age ranged from 1 year 3 months to 14 years. The time from onset to diagnosis was 3 months to 3 years. The follow-up duration was 6 months to 6 years. All the 4 cases had cough, 2 cases had tachypnea and wheezing, 1 case had expectoration, 1 case had chest pain, 1 case had decreased activity tolerance and weight loss. Rales in 2 cases and hypoxemia in 1 case. Pulmonary high resolution CT showed diffuse distribution, involvement of both lungs in 3 cases, and single lung combined migration in 1 case.Three cases showed ground-glass opacity, consolidation, patchy or fibrous strips, and 1 case presented air bronchogram and "reversed halo sign". All the 4 cases were performed thoracoscopic lung biopsy, and the pathological findings showed cellulose exudate or small nodules filled with granulation tissue or fibroblasts in the alveolar cavity and small airways, and 1 case was Masson corpuscle positive. Three patients achieved remission after glucocorticoid therapy. Spontaneous remission without treatment was seen in 1 patient.Two cases were followed up for 17 months and 6 years, respectively, who had excellent outcome. Conclusions: The manifestations of COP in children include cough, expectoration and chest pain. Infants and young children may have tachypnea and wheezing. The most common chest CT findings are diffuse distribution of ground-glass opacity, patchy and consolidation in both lungs. Diagnosis should depend on pathological examination. The effect of glucocorticoid therapy is good.

Heterogeneity of determining disease severity, clinical course and outcomes in systemic sclerosis-associated interstitial lung disease: a systematic literature review

Objective The course of systemic sclerosis-associated interstitial lung disease (SSc-ILD) is highly variable and different from continuously progressive idiopathic pulmonary fibrosis (IPF). Most proposed definitions of progressive pulmonary fibrosis or...

Is JAK inhibition an option in the treatment of interstitial lung disease in rheumatoid arthritis?

A69-year-old male patient with seropositive erosive rheumatoid arthritis (RA) presented to our clinic due to progressive dyspnea. High-resolution computed tomography (HRCT) and immunological bronchioalveolar lavage revealed ground-glass opacities and alymphocytic alveolitis caused by interstitial lung disease (ILD) in RA. Considering previous forms of treatment, disease-modifying antirheumatic drug (DMARD) treatment was switched to tofacitinib. Tofacitinib treatment demonstrated a33% reduction in ground-glass opacities by artificial intelligence-based quantification of pulmonary HRCT over the course of 6 months, which was associated with an improvement in dyspnea symptoms. In conclusion, tofacitinib represents an effective anti-inflammatory therapeutic option in the treatment of RA-ILD.

Retrospective comparative study of the efficacy of JAK inhibitor (tofacitinib) in the treatment of systemic sclerosis-associated interstitial lung disease.

The oral Janus kinases inhibitor (JAKi) has improved the management of skin manifestations in systemic sclerosis (SSc), and our study aimed to explore the efficacy of non-selective JAKi tofacitinib in ameliorating interstitial lung disease (ILD) in the patients with SSc. The hospitalization data of the SSc-ILD patients from April 2019 to April 2021 were collected, and the changes of pulmonary function and the radiological findings in pulmonary high-resolution CT (HRCT) from the 9 patients who received tofacitinib for at least 6months and a matched group of 35 SSc-ILD patients treated with conventional immunosuppressants or glucocorticoids, were compared and analyzed. There were no significant differences in demographic data and clinical characteristics between the tofacitinib-treated group (tofa-group) and the matched group. However, in the tofa-group, the changes in serum lactate dehydrogenase (LDH) concentration and serum interleukin-6 levels were significantly lower than those in the matched group. Moreover, the tofa-group showed amelioration in decreased diffusing capacity of the lung for carbon monoxide (DLCO) (62.05 ± 9.47 vs. 66.61 ± 12.39, p = 0.046), reductions in ground-glass attenuation involvement (1.00 ± 0.86 vs. 0.33 ± 0.50, p = 0.024) and irregular pleural thickening (1.33 ± 0.50 vs. 0.67 ± 0.51, p = 0.004) in pulmonary HRCTs, alleviated modified Rodnan skin score (mRSS) of skin sclerosis (9.22 ± 3.81 vs. 7.11 ± 3.92, p = 0.048), and reduced HRCT scores of pulmonary fibrosis (15.00 ± 3.87 vs. 12.66 ± 4.92, p = 0.009). Logistic regression analysis showed that the involvement of ground-glass attenuation (OR 11.43) and the add-on therapy of tofacitinib (OR 9.98) were the relevant factors in the amelioration of HRCT. Our results indicate that the use of JAKi (tofacitinib) may be relevant to significant improvement of the sclerosis and early radiological abnormalities in SSc-ILD patients. Further studies are needed to confirm these findings and to explore its efficacy more precisely. Key Points • The currently available therapies for SSc-ILD have limited therapeutic benefits. • The add-on therapy of the oral JAK inhibitor is available in the real world. • The tofacitinib was promising in the improvement of the sclerosis and early radiological abnormalities in SSc-ILD patients.

Clinical characteristics of myositis patients with isolated anti-U1 ribonucleoprotein antibody resemble immune-mediated necrotizing myopathy.

Anti-U1 ribonucleoprotein (U1RNP) antibodies were associated with connective tissue diseases (CTDs), but the clinical characteristics of this antibody in Chinese myositis patients have not been studied. We aim to analyze the clinical features of myositis patients who test positive for anti-U1RNP antibodies and delineate a subgroup of myositis. This is a retrospective cohort study. We reviewed the clinical data of myositis patients with anti-U1RNP antibodies and compared them to those with anti-signal recognition particle (SRP) and hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibody-associated immune-mediated necrotizing myopathy (IMNM). A total of 30 adult cases were identified; median age was 47.5 years and 24 (80%) were females, and 12 patients did not coexist with myositis-specific antibodies (MSAs) (isolated anti-U1RNP). The serum creatine kinase (CK) was significantly higher in patients with isolated anti-U1RNP (2182.5 U/L versus 289 U/L, p = 0.01), and the number of patients with CK > 2000 U/L was higher compared to that in anti-U1RNP antibody patients coexisting with MSAs (66.7% versus 16.7%, p = 0.009). The prevalence of IMNM in patients' muscle pathology with isolated anti-U1RNP was significantly higher (75%, p = 0.003). Skin rashes were less common in isolated anti-U1RNP group (p < 0.05). Of the 25 individuals with available pulmonary high-resolution CT (HRCT), 14 (56%) were diagnosed with interstitial lung disease (ILD). The incidence of muscular weakness, dysphagia, or levels of CK was not different between the isolated anti-U1RNP antibody individuals and the anti-HMGCR or SRP-IMNM groups (p > 0.05). But the frequency of Raynaud's phenomenon, arthritis, and membrane attack complex (MAC) deposits in myositis patients with isolated anti-U1RNP antibodies were higher than in anti-HMGCR and SRP-IMNM (all p < 0.005). There was no difference between anti-U1RNP patients with and without Ro-52 (p > 0.05). Isolated anti-U1RNP individuals showed marked improvements in muscle strength, and the remission rate in 1 and 2 years was significantly higher than that in anti-HMGCR and SRP-IMNM (p < 0.05). The clinical and pathological features of myositis patients with isolated anti-U1RNP antibodies were similar to IMNM. Arthritis and ILD are the most common extramuscular clinical features. Most respond well to treatment and have a good prognosis.

The outcome of pulmonary function tests and high-resolution computed tomography of chest in post-coronavirus disease 2019-confirmed cases after 3 months of recovery

Background Coronavirus disease (COVID-19) is a recently prevalent infectious disease that is caused by a virus from the coronavirus family and causes acute respiratory syndrome. It is a pandemic catastrophe that has affected more than 60 million people around the world and has caused about 1.5 million deaths, as reported by the WHO. This disease affects the respiratory system and leads to different forms of symptoms and signs. Pneumonia is a common cause for hospitalization, with most patients treated in hospital wards and others requiring ICU. Although the number of complete recoveries from COVID-19 has increased, there is still concern about complications associated with the disease that appear after recovery. The studies that have looked at past types and other forms of coronavirus epidemics, such as SARS have shown that some cases had respiratory complications from the infection after being full recovered, as 36 and 30% of the entire study population had clinical and high-resolution computed tomography (HRCT) changes at 3 and 6 months after recovery, respectively. Mostly, the abnormalities seen in pulmonary function test (PFT) results are sequelae of diffusion capacity defect. In recovered cases of Middle East respiratory syndrome, 36% of patients showed HRCT sequelae at follow-up of 6 weeks, because of fibrosis. Data on COVID-19 indicate that prolonged disease and persistent symptoms show post-PFT affection and follow-up radiographic changes after recovery from COVID-19 as interstitial pulmonary changes and a degree of pulmonary vasculopathy. In recovered cases of COVID-19, capacity of diffusion is the commonest defect in lung function, followed by the restrictive pattern defects on spirometry; both are related to the degree of severity of pneumonic COVID-19. PFTs (involving spirometry as well as diffusion capacity) are considered as routine follow-up examinations for some of the recovered cases, especially severe cases. Rehabilitation programs of the respiratory system are an option strategy that might be considered. This study aims to show changes in pulmonary function and HRCT of chest in post-COVID-19-infected patients to detect long-term effects on the lungs after 3 months as obstructive or restrictive, or both, lung diseases. Patients and methods The study was conducted on 100 confirmed PCR-positive COVID-19 cases that were admitted to Ain Shams University Isolation Hospitals, and the follow-up was performed in the outpatient clinic. PCR samples (Combined nasopharyngeal and oropharyngeal swab) were taken after 3 months from discharge of patients above the age of 18 years who become negative with clinical improvement. PFT [spirometry and diffusion for carbon monoxide (DLCO)] and chest HRCT were done. All patients’ clinical data were recorded, and CT chest imaging data of these patients were correlated with the clinical data. Results A total of 100 patients were included in this study, where males represented 58% and female represented 42%. The mean±SD age of cases in this study was 45.05 ± 11.80 years and ranged from 20 to 79 years. CT chest severity score (SS) of abnormality in COVID-19-infectedd patients based on HRCT chest findings before and after 3 months from treatment showed a highly significant correlation (P=0.000). The results of PFT in the studied group after 3 months of discharge showed restrictive pattern in 14.9%, obstructive pattern in 17.8%, and both obstructive and restrictive patterns in 5.9% of the total number of cases. There was a significant correlation between DLCO abnormality findings and age of studied group (P=0.032), a significant correlation between abnormality findings on PFT and HRCT chest SS after discharge of the studied group (P&lt;0.001). There was a significant correlation between abnormality findings of DLCO and HRCT chest SS after 3 months of the studied group (P=0.000) and before treatment (P=0.001), whereas there was no significant correlation between other findings of PFT and HRCT chest SS after 3 months and before. There was a significant correlation between HRCT chest SS of the studied group and their age after (P=0.003) and before treatment (P=0.000). This cohort study provided the long-term dynamic sequelae and variations of lung function after hospital discharge. Conclusion DLCO-predicted value detected at 3 months is the most important factor associated with severe respiratory COVID-19, which is identified as the cause of impairment of any ordinary basic activity of patients in the stage of postrecovery from COVID-19. The radiological chest findings must be analyzed in conjunction with other functional parameters to provide accurate assessment of the disease. These findings highlight the significance of a comprehensive follow-up after severe and critical COVID-19 infection, with appropriate management of pulmonary consequences. Patients after discharge and on follow-up in outpatient clinics in the post-COVID-19 stage should be strictly followed up with PFTs. Further studies with longer follow-up periods might be needed.

Post-COVID-19 tomographic abnormalities.

BACKGROUND: The prevalence of persistent respiratory symptoms tends to be low in patients with a longer recovery time after COVID-19. However, some patients may present persistent pulmonary abnormalities.OBJECTIVE: To evaluate the prevalence of tomographic abnormalities 90 days after symptom onset in patients with COVID-19 and compare two chest high-resolution computed tomography (HRCT) analysis techniques.METHODS: A multicentre study of patients hospitalised with COVID-19 having oxygen saturation <93% on room air at hospital admission were evaluated using pulmonary function and HRCT scans 90 days after symptom onset. The images were evaluated by two thoracic radiologists, and were assessed using software that automatically quantified the extent of pulmonary abnormalities.RESULTS: Of the 91 patients included, 81% had at least one pulmonary lobe with abnormalities 90 days after discharge (84% were identified using the automated algorithm). Ground-glass opacities (76%) and parenchymal bands (65%) were the predominant abnormalities. Both chest HRCT technical assessments presented high sensitivity (95.9%) and positive predictive value (92%), with a statistically significant correlation at baseline (R = 0.80) and after 90 days (R = 0.36).CONCLUSION: The prevalence of pulmonary abnormalities on chest HRCT 90 days after symptom onset due to COVID-19 was high; both technical assessments can be used to analyse the images.

Assessing the diagnostic value of a potential screening tool for detecting early interstitial lung disease at the onset of inflammatory rheumatic diseases

BackgroundInterstitial lung disease (ILD) is a severe pulmonary complication in inflammatory rheumatic diseases (IRD) and associated with significantly increased morbidity and mortality. That is why ILD screening at a very early stage, at the onset of IRD, is essential. The objective of the present study was to evaluate the diagnostic value and utility of a stepwise approach as a potential ILD screening tool in patients with newly diagnosed IRD.MethodsConsecutively, 167 IRD patients were enrolled. To homogenize the study cohort, an age and gender matching was performed. The case-control study included 126 patients with new onset of IRD (mainly connective tissue diseases [CTD], small vessel vasculitis, and myositis). We applied a stepwise screening algorithm in which all patients underwent pulmonary function testing (PFT) and/or additional chest radiography. If there was at least one abnormal finding, pulmonary high-resolution computed tomography (HRCT) was subsequently performed.ResultsWith our stepwise diagnostic approach, we identified 63 IRD patients with ILD (ILD group) and 63 IRD patients without ILD (non-ILD group). A reduced diffusing capacity for carbon monoxide (DLCO) < 80% showed a sensitivity of 83.6% and a specificity of 45.8% compared to chest X-ray with 64.2% and 73.6%, respectively, in detecting ILD. The combination of reduced DLCO and chest X-ray revealed a sensitivity of 95.2% and a specificity of 38.7%. The highest sensitivity (95.2%) and specificity (77.4%) were observed for the combination of reduced DLCO, chest X-ray, and pulmonary HRCT. The most common pulmonary abnormalities on HRCT were ground-glass opacities (GGO; 36.5%), followed by non-specific interstitial pneumonia (NSIP; 31.8%) and usual interstitial pneumonia (UIP; 9.5%).ConclusionsThe combination of reduced DLCO (< 80%), chest X-ray, and pulmonary HRCT yielded the highest sensitivity and specificity in detecting ILD at the onset of IRD. Therefore, this stepwise approach could be a new screening algorithm to identify IRD patients with pulmonary involvement already at the time of the initial IRD diagnosis.

Spectrum of Typical and Atypical Pulmonary CT Imaging Findings of COVID-19 Infection: A Retrospective Study.

Coronavirus disease 2019 (COVID-19) is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The first known case was identified in Wuhan, China, in December 2019. It was declared a public health emergency by WHO in January 2020. The definitive diagnostic test for COVID-19 is a real time polymerase chain reaction test (RT-PCR) which is highly specific, but sensitivity is variable. COVID-19 typically presents clinically with respiratory and systemic symptoms. The majority of the infected patients are asymptomatic during the course of the disease, which we have not included in our study. Imaging findings on high-resolution computed tomography (HRCT) chest are important to diagnose the disease in early stage, for treatment planning and to predict the patient prognosis. The purpose of our study was to characterize typical and atypical pulmonary and extra-pulmonary HRCT findings in patients with COVID-19 infection and to help in the management of patients. In this retrospective study, we have included 70 patients who had undergone HRCT examination of the chest in the Radiodiagnosis Department, Maharishi Markandeshwar Medical College, Kumarhatti, Solan, Himachal Pradesh, India. The HRCT findings of the chest of these patients in the study will be evaluated and data will be statistically analyzed.

Underdetection of Interstitial Lung Disease in Juvenile Systemic Sclerosis.

Utilizing data obtained from a prospective, international, juvenile systemic sclerosis (SSc) cohort, the present study was undertaken to determine if pulmonary screening with forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLco) is sufficient to assess the presence of interstitial lung disease (ILD) in comparison to high-resolution computed tomography (HRCT) in juvenile SSc. The juvenile SSc cohort database was queried for patients enrolled from January 2008 to January 2020 with recorded pulmonary function tests (PFTs) parameters and HRCT to determine the discriminatory properties of PFT parameters, FVC, and DLco in detecting ILD. Eighty-six juvenile SSc patients had both computed tomography imaging and FVC values for direct comparison. Using findings on HRCT as the standard measure of ILD presence, the sensitivity of FVC in detecting ILD in juvenile SSc was only 40%, the specificity was 77%, and area under the curve (AUC) was 0.58. Fifty-eight juvenile SSc patients had both CT imaging and DLco values for comparison. The sensitivity of DLco in detecting ILD was 76%, the specificity was 70%, and AUC was 0.73. The performance of PFTs in juvenile SSc to detect underlying ILD was quite limited. Specifically, the FVC, which is one of the main clinical parameters in adult SSc to detect and monitor ILD, would miss ~60% of children who had ILD changes on their accompanying HRCT. The DLco was more sensitive in detecting potential abnormalities on HRCT, but with less specificity than the FVC. These results support the use of HRCT in tandem with PFTs for the screening of ILD in juvenile SSc.

Late-Onset, Noninfectious Pulmonary Complications following Allogeneic Hematopoietic Stem Cell Transplantation: A Nationwide Cohort Study of Long-Term Survivors

Background: Survivors of allogeneic hematopoietic stem cell transplantation (allo-HSCT) are at risk for pulmonary adverse events. Data on late-onset noninfectious pulmonary complications in long-term adult survivors of allo-HSCT are limited and incomplete. Objectives: This study aimed (1) to determine occurrence and degree of pulmonary sequelae in adult survivors of allo-HSCT and (2) to identify associations between pulmonary function, high-resolution CT (HRCT), and clinical characteristics. Method: In a nationwide, single-center cross-sectional study, 103 survivors (aged median [range] 35 [17–58] years, 53% females) were examined 17 (6–32) years after allo-HSCT and compared with healthy controls (n = 105). Methods included pulmonary function tests and HRCT. Results: Chronic graft-versus-host disease was diagnosed in 33% of survivors, including 12% with bronchiolitis obliterans syndrome (BOS). Mean lung volumes (TLC, FVC, and FEV₁) and gas diffusing capacity were >80% of predicted for the survivors as a group, but significantly lower than in healthy controls. Pathological HRCT findings were detected in 48% of the survivors (71% airways disease, 35% interstitial lung disease, and 24% apical subpleural interstitial thickening). Air trapping (%) on HRCT correlated with % predicted FEV₁, p < 0.001. In a multiple logistic regression model, both BOS and pathological findings on HRCT were associated with chemotherapy prior to allo-HSCT, p < 0.05. Conclusions: Long-term allo-HSCT survivors had significantly lower pulmonary function than age- and gender-matched healthy controls and nearly half had pathological findings on HRCT. Longitudinal data will determine if pulmonary sequelae will remain stable or progress. We recommend lifelong monitoring of pulmonary function in allo-HSCT survivors. HRCT provides additional information, but is not suited for surveillance.

A Computed Tomography Radiomics-Based Prediction Model on Interstitial Lung Disease in Anti-MDA5-Positive Dermatomyositis

Objectives: Anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated interstitial lung disease (MDA5+ DM-ILD) is a life-threatening disease. The current study aimed to quantitatively assess the pulmonary high-resolution computed tomography (HRCT) images of MDA5+ DM-ILD by applying the radiomics approach and establish a multidimensional risk prediction model for the 6-month mortality.Methods: This retrospective study was conducted in 228 patients from two centers, namely, a derivation cohort and a longitudinal internal validation cohort in Renji Hospital, as well as an external validation cohort in Guangzhou. The derivation cohort was randomly divided into training and testing sets. The primary outcome was 6-month all-cause mortality since the time of admission. Baseline pulmonary HRCT images were quantitatively analyzed by radiomics approach, and a radiomic score (Rad-score) was generated. Clinical predictors selected by univariable Cox regression were further incorporated with the Rad-score, to enhance the prediction performance of the final model (Rad-score plus model). In parallel, an idiopathic pulmonary fibrosis (IPF)-based visual CT score and ILD-GAP score were calculated as comparators.Results: The Rad-score was significantly associated with the 6-month mortality, outperformed the traditional visual score and ILD-GAP score. The Rad-score plus model was successfully developed to predict the 6-month mortality, with C-index values of 0.88 [95% confidence interval (CI), 0.79–0.96] in the training set (n = 121), 0.88 (95%CI, 0.71–1.0) in the testing set (n = 31), 0.83 (95%CI, 0.68–0.98) in the internal validation cohort (n = 44), and 0.84 (95%CI, 0.64–1.0) in the external validation cohort (n = 32).Conclusions: The radiomic feature was an independent and reliable prognostic predictor for MDA5+ DM-ILD.

Follow-up on the therapeutic effects of a budesonide, azithromycin, montelukast, and acetylcysteine (BAMA) regimen in children with post-infectious bronchiolitis obliterans.

BackgroundPost-infectious bronchiolitis obliterans (PIBO) is a rare, severe chronic lung disease without optimal treatment. Currently, treatment in children mainly relies on systemic corticosteroids, but long-term use of these drugs may lead to adverse reactions. This study aimed to evaluate the short-term efficacy of the budesonide, azithromycin, montelukast, and acetylcysteine (BAMA) regimen in paediatric PIBO patients and whether it can reduce systemic corticosteroid use.MethodsThis was a prospective study. From June 2017 to July 2020, patients diagnosed with PIBO at Yuying Children’s Hospital of Wenzhou Medical University were treated with the BAMA regimen for 3 months. Methylprednisolone was added only when the clinical manifestations did not improve or deteriorated. All patients were followed up every 2 to 4 weeks, and changes in clinical symptoms were recorded. Pulmonary function tests and high-resolution computed tomography (HRCT) were performed, and systemic corticosteroid use was recorded after the 3-month follow-up.ResultsA total of 75 patients with PIBO were included; overall, 54 patients completed the course of treatment. After treatment, the respiratory manifestations were improved in 37 patients (68.5%), but 4 patients (7.4%) showed decreased exercise tolerance, and 2 patients (3.7%) were readmitted to the hospital. Additionally, 17 (56.7%) of the 30 patients whose pulmonary function was re-examined showed improvement, and 28 (77.8%) of the 36 patients who underwent HRCT showed marked improvement. Importantly, 20 patients (54.1%) received systemic corticosteroids for 2 weeks or less, while 31.5% of patients used no corticosteroids.ConclusionsThe BAMA regimen effectively relieved clinical symptoms and signs of PIBO in children, improved pulmonary function and HRCT manifestations, and reduced the use of systemic corticosteroids.

POS0842 LUNG ULTRASOUND TO ASSESS THE SEVERITY OF INTERSTITIAL LUNG DISEASE IN SYSTEMIC SCLEROSIS

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

Utility of lung ultrasound to identify interstitial lung disease: An observational study based on the STROBE guidelines.

Lung ultrasound (LUS) has recently been used to identify interstitial lung disease (ILD). However, data on the role of LUS in the detection of ILD remain limited. The aim of this study was to investigate the diagnostic value of LUS compared with high-resolution computed tomography (HRCT) in patients with ILD.The retrospective study was carried out by reviewing the medical records of patients with respiratory signs and symptoms discharged from the respiratory ward. Only patients with suspected ILD who underwent HRCT and LUS within a week were selected. ILD was identified with a semi-quantitative score of B-lines >5 and a Warrick score >0 points. The endpoints of LUS in diagnosing ILD (i.e., sensitivity, specificity, positive likelihood ratio [PLR], negative likelihood ratio [NLR], positive predictive value [PPV], and negative predictive value [NPV], and receiver operating characteristic [ROC] curve) was compared with that of HRCT. The reference standard used for the diagnosis of ILD was based on history, clinical findings and examination, and laboratory and instrumental tests, including pulmonary function tests, lung histopathology, and HRCT (without LUS findings).The final clinical diagnosis of ILD was 55 in 66 patients with suspected ILD. HRCT was positive in 55 patients, whereas LUS detected ILD in 51 patients. Four patients with negative LUS findings were positive on HRCT. The results showed 93% sensitivity, 73% specificity, 3.40 PLR, 0.10 NLR, 94% PPV, and 67% NPV for LUS, whereas 100% sensitivity, 82% specificity, 5.49 PLR, 0.01 NLR, 97% PPV, and 100% NPV for HRCT. Comparison of the 2 ROC curves revealed significant difference in the diagnostic value of the 2 methods for the diagnosis of ILD (P = .048).Our results indicated that LUS is a useful technique to identify ILD. Considering its non-radiation, portable and non-invasive advantages, LUS should be recommended as a valuable screening tool in patients with suspected ILD.

Pulmonary findings on high-resolution computed tomography in Takayasu arteritis.

This study aimed to describe pulmonary high-resolution CT (HRCT) findings in Takayasu arteritis (TA) and to determine possible causes. A total of 243 TA patients were enrolled from a prospective cohort after excluding patients with other pulmonary disorders or incomplete data. Patients were divided into two groups: those with normal lung HRCT and those with abnormal lung HRCT. Clinical characteristics were compared between groups and binary logistic regression analysis was applied to identify possible causes of the lung lesions. Follow-up HRCT (obtained in 64 patients) was analysed to study changes in pulmonary lesions after treatment. Of the 243 patients, 107 (44.0%) had normal lung HRCT while 136 (56.0%) had abnormal lung HRCT, including stripe opacity (60.3%), nodules (44.9%), patchy opacity (25.0%), pleural thickening (15.4%), pleural effusion (10.3%), ground-glass opacity (8.1%), pulmonary infarction (6.6%), mosaic attenuation (4.4%), bronchiectasis (3.7%) and pulmonary oedema (2.2%). Patients with abnormal HRCT were significantly more likely to have type II arterial involvement (25% vs 12.2%, P = 0.04), pulmonary arterial involvement (PAI; 21.3% vs 5.6%, P < 0.001), pulmonary hypertension (20.6% vs 8.4%, P = 0.01) and abnormal heart function (27.9% vs 7.6%, P < 0.001). Logistic regression analysis demonstrated that PAI, worsened heart function and age were associated with presence of pulmonary lesions. Pulmonary infarction, pleural effusion and patchy opacities improved partially after treatment. Pulmonary lesions are not rare in patients with TA. Age, PAI and worsened heart function are potential risk factors for presence of pulmonary lesions in TA.