High-resolution Computed Tomography Scores Research Articles

Bronchoscopy-guided antimicrobial therapy for cystic fibrosis.

Bronchoscopy-guided antimicrobial therapy for cystic fibrosis.

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.

Risk factors for progression of pulmonary fibrosis: a single-centered, retrospective study.

This study aimed to identify clinical characteristics associated with the prevalence of progressive pulmonary fibrosis (PPF) in interstitial lung disease (ILD) and to develop a prognostic nomogram model for clinical use. In this single-centered, retrospective study, we enrolled ILD patients with relatively comprehensive clinical data and assessed the incidence of PPF within a year using collected demographics, laboratory data, high-resolution computed tomography (HRCT), and pulmonary function test (PFT) results. We used a training cohort of ILD patients to identify early predictors of PPF and then validated them in an internal validation cohort and subsets of ILD patients using a multivariable logistic regression analysis. A prognostic nomogram was formulated based on these predictors, and the accuracy and efficiency were evaluated using the area under the receiver operating characteristic curve (AUC), calibration plot, and decision curve analysis (DCA). Among the enrolled patients, 120 (39.09%) cases had connective tissue disease-associated interstitial lung disease (CTD-ILD), 115 (37.46%) had non-idiopathic pulmonary fibrosis idiopathic interstitial pneumonia (non-IPF IIP), and 35 (11.4%) had hypersensitivity pneumonitis (HP). Overall, 118 (38.4%) cases experienced pulmonary fibrosis progression. We found that baseline DLco% pred (OR 0.92; 95% CI, 8.93-0.95) was a protective factor for ILD progression, whereas combined pneumonia (OR 4.57; 95% CI, 1.24-18.43), modified Medical Research Council dyspnea score (mMRC) (OR 4.9; 95% CI, 2.8-9.5), and high-resolution computed tomography (HRCT) score (OR 1.22; 95% CI, 1.07-1.42) were independent risk factors for PPF. The AUC of the proposed nomogram in the development cohort was 0.96 (95% CI, 0.94, 0.98), and the calibration plot showed good agreement between the predicted and observed incidence of PPF (Hosmer-Lemeshow test: P = 0.86). ILD patients with combined pneumonia, low baseline DLco% pred, high mMRC marks, and high HRCT scores were at higher risk of progression. This nomogram demonstrated good discrimination and calibration, indicating its potential utility for clinical practice.

Classification of COVID-19 patients from HRCT score prediction in CT images using transfer learning approach

COVID-19 had a huge impact on patients and medical systems all around the world. Computed tomography (CT) images can effectively complement the reverse transcription-polymerase chain reaction testing (RT-PCR) and offer results much faster than RT-PCR test which assists to prevent spread of COVID-19. Various deep learning models have been recently proposed for COVID-19 screening in CT scans as a tool to automate and help the diagnosis, but consisting of some benefits and limitations. Some of the reasons for this are: (i) training the data with largely unbalanced dataset and (ii) training the models with datasets having all similar CT images which leads to overfitting. In this work, we proposed a method to use multiple models to classify COVID-19 positive or negative which are trained using transfer learning techniques. In addition to classifying, if a person is COVID-19 positive or negative, we have also calculated the high-resolution computed tomography (HRCT) score or CT score to find the severity of infection with the help of image segmentation techniques, which assist in identifying the preliminary prognosis of the patient, and take necessary preventive measures.

Clinical profile of COVID-19 patients and factors impacting mortality in a rural tertiary care center of Solapur

Background: A retrospective and cohort study was conducted in our center to study the parameters among surviving and non-surviving patients that have a significant impact on in hospital mortality. Along with this, demographic and clinical profile was also studied. Aims and Objectives: The aim of this study was to evaluate clinical profile of COVID-19 patients and factors impacting mortality. Materials and Methods: The present study is a single-center, retrospective, and cohort study done on COVID-19 positive (by real-time polymerase chain reaction) patients admitted between May 2019 and June 2021 in the intensive care unit (ICU). Of the total 1694 patients, 422 required ICU treatment and the remaining 1272 patients were treated in wards and rooms and discharged home. The analysis was based on demographic, clinical, associated comorbidities, laboratory and radiological data, and treatment modalities used. Outcome data were analyzed by regression analysis and the Kaplan–Meir survival plot was used to calculate mortality trend. Results: Of the 422 patients, 158 (37.4%) died and 264 (62.6%) survived. Males predominated with 297/422 (70.4%). ICU admissions comprised 115/158 (72.8%) of the total deaths. Fever (88%), dyspnea (78.48), and cough (67.1%) were the most common symptoms in non-survival group. Hypertension (43.7%) and diabetes (37.3%) were the most common comorbidities. High oxygen (&gt;10 L/min) (P&lt;0.0001), invasive ventilation (P&lt;0.0001), noradrenaline infusion P&lt;0.0001), renal replacement therapy (P&lt;0.0001) and tocilizumab requirement (P&lt;0.0001), procalcitonin (P&lt;0.009) and interleukin-6 (P&lt;0.004) acute respiratory distress syndrome (ARDS) with septic shock, and multiorgan failure (P&lt;0.0001), all indicated higher risk of death. Conclusion: Mortality was 37.4% in our study. Elderly age (&gt;60 years), high serum ferritin (&gt;931.9), and severe disease with a high resolution computed tomography score (&gt;15), ARDS with septic complications requiring invasive ventilation and vasopressor support, are associated with increased mortality in these patients.

Progression of radiographic fibrosis in rheumatoid arthritis-associated interstitial lung disease.

Preclinical interstitial lung disease (pILD) may represent the early stages of rheumatoid arthritis-associated interstitial lung disease (RA-ILD). However, the characteristics, clinical outcomes, and risk factors associated with fibrosis progression in RA-ILD, including pILD and ILD, remain poorly understood. Baseline data were compared between patients with RA-ILD and those with RA alone. Multivariate logistic regression and Cox regression analyses were performed to identify risk factors associated with the prevalence and imaging progression of RA-ILD, respectively. Among the 371 enrolled RA patients, 32.3% had RA-ILD. Multiple logistic regression analyses identified age over 60.0 years (OR 2.22), smoking (OR 2.09), diabetes mellitus (DM) (OR 3.09), mixed connective tissue disease (MCTD) (OR 2.98), serum lactate dehydrogenase (LDH) levels exceeding 250.0 U/L (OR 6.73), and positive anti-cyclic citrullinated peptide (anti-CCP) antibody (OR 2.06) as independent risk factors for RA-ILD (p< 0.05 or 0.01). Among the 98 RA-ILD patients who underwent follow-up for a median duration of 19.1 months, 51.0% demonstrated fibrotic progression on high-resolution computed tomography (HRCT). Multiple Cox regression analysis identified DM (HR 2.03), Disease Activity Score in 28 joints-Erythrocyte Sedimentation Rate (DAS28-ESR) greater than 5.1 (HR 2.21), and baseline HRCT scores exceeding 5.0 (HR 2.30) as independent risk factors for fibrosis progression in RA-ILD (p< 0.05 or 0.01). Nearly one-third of RA patients in this cohort had prevalent pILD or ILD, and half of them demonstrated imaging progression during follow-up. DM, higher DAS28-ESR, and advanced HRCT scores were identified as independent risk factors for progressive fibrosis in RA-ILD.

The additional treatment value of immunoglobulin for the treatment of rheumatoid arthritis complicated with interstitial lung disease: A propensity score-matched pilot study.

To explore the additional treatment value of intravenous immunoglobulin injections for treating interstitial lung disease (ILD) caused by rheumatoid arthritis (RA). This pilot study included patients with RA-ILD. The RA-ILD patients were grouped by treatment agents: traditional agents (disease-modifying antirheumatic drugs, [D]MARDs] and glucocorticoids) and traditional agents plus immunoglobulin. A propensity matching score (PSM) was performed to balance the bias of baseline characteristics. The treatment efficacy and safety indicators were analyzed and compared between the two groups. In total, 134 patients were included in this study. After PSM, 80 patients were finally included, with 40 in each group. The immunoglobulin group consisted of 12 men and 28 women with a mean age of 51.5 ± 8.4 years (22-75 years). The control group included 13 men and 27 women, with a mean age of 50.6 ± 8.2 years (25-74 years). The chronic obstructive pulmonary disease assessment test score in the immunoglobulin group was statistically lower after treatment than in the control group (19.1 ± 3.3 vs. 17.7 ± 3.4, p = .03). The 6-min walking distance (364.4 ± 54.3 vs. 332.3 ± 55.1, p = .04) and forced vital capacity (78.8 ± 12.6 vs. 66.6 ± 11.2, p = .05) were statistically higher in the immunoglobulin group. The high-resolution computed tomography score and erythrocyte sedimentation rate were both statistically lower in the immunoglobulin group (both p < .05). The adverse event rate did not differ between the two groups (p = .61). The additional use of immunoglobulin intravenous injection is effective for the treatment of RA-ILD with no additional adverse effects.

Circulating Biomarkers and Disease Activity in Systemic Sclerosis-Associated Interstitial Lung Disease

Background: Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is a major cause of death in SSc patients. Several studies have reported that serum anti-Scl-70, Krebs von Lungren-6 (KL-6), and surfactant protein D (SP-D) levels are associated with the presence and progression of ILD in SSc patients. Objective: To examine the correlation between levels of these serum biomarkers and disease severity determined by baseline dyspnea index (BDI), high-resolution computed tomography (HRCT) score, and pulmonary function tests. Materials and Methods: The present study was a single-center, cross-sectional study. Serum anti-Scl-70, KL-6, and SP-D from 20 SSc-ILD patients and five non-ILD subjects were measured. The BDI, HRCT score, and pulmonary function tests were used to assess the severity of ILD in SSc-ILD patients. HRCT abnormalities, including ground-glass opacity (GGO), fibrosis, and honeycombing, were scored by using the semi-quantitative scoring system. Results: Serum anti-Scl-70, KL-6, and SP-D in SSc-ILD patients were significantly higher than those in non-ILD subjects. There was a moderate correlation between diffusing capacity for carbon monoxide (DLCO) and serum KL-6 levels (r=–0.551, p=0.022), while the pulmonary fibrosis (PF) score exhibited a strong correlation with serum KL-6 levels (r=0.630, p=0.003). The PF score had a moderate negative correlation with forced vital capacity (FVC) (r=–0.515, p=0.034) and a strong negative correlation with total lung capacity (TLC) and DLCO (r=–0.625, p=0.007, and r=–0.762, p&lt;0.001, respectively). Conclusion: The levels of serum KL-6, and SP-D are elevated in SSc-ILD patients. Serum KL-6 may be a useful non-invasive biomarker for the disease severity, as determined by DLCO and the extent of fibrosis on HRCT, in patients with SSc-ILD. Trial registration: Thai Clinical Trials Registry, TCTR20200314001, registered 13 March 2020, retrospectively registered at http://www.thaiclinicaltrials.org/show/TCTR20200314001 Keywords: Scleroderma; Systemic sclerosis; Interstitial pneumonia; Interstitial lung disease; Fibrosis; Biomarker

Therapeutic effects of thalidomide on patients with systemic sclerosis-associated interstitial lung disease.

To evaluate the clinical efficacy of thalidomide in patients with systemic sclerosis-associated interstitial lung disease. Ninety-six systemic sclerosis-associated interstitial lung disease patients who received basic glucocorticoid treatment and admitted between 2016 and 2020 were included in this study, including 48 cases in the thalidomide group (combination of thalidomide and cyclophosphamide) and 48 cases in control group (cyclophosphamide monotherapy). Evaluation items included clinical symptoms, modified Rodnan skin score, pulmonary function test, chest high-resolution computed tomography scores, and adverse effects between two groups after 24 weeks of treatment. Remarkable improvements in several aspects were found in the thalidomide group, including modified Rodnan skin score, expiratory dyspnea score, cough visual analog scale score, total ground-glass opacity score, and total interstitial lung disease score. Compared to the control group, improvements in the thalidomide group were found, such as significantly decreased cough visual analog scale score and expectoration; increased number of platelets; improved pulmonary fibrosis (p = 0.056), and reduced carbon monoxide diffusing capacity (p = 0.053). There were no statistically significant differences in the expiratory dyspnea score and predicted forced vital capacity between the two groups. Patients who experienced at least one adverse event in the control group and thalidomide group were 33.3% and 64.6% (p = 0.002); while those with serious adverse events were 8.3% versus 12.5% (p = 0.504). Venous thrombosis was found in one case in the thalidomide group. Thalidomide combined with cyclophosphamide can improve the symptoms of cough and expectoration in patients with systemic sclerosis-associated interstitial lung disease, and may slightly delay the progression of pulmonary fibrosis, but with the possibility of an increased risk of adverse events.

Serum sCD25 is an indicator for rheumatoid arthritis-associated interstitial lung disease.

CD25 (IL-2Rα) is one of IL-2 receptor's polypeptide subunits, and its soluble form is increased in patients with various inflammatory or autoimmune diseases. This study aimed to evaluate the clinical correlation of serum soluble CD25 (sCD25) with interstitial lung disease (ILD) in rheumatoid arthritis (RA) patients. 294 RA patients, including 72 in the discovery cohort (15 patients with ILD, 57 patients without ILD), 222 in the validation cohort (41 patients with ILD and 181 patients without ILD), and 58 healthy controls (HCs) were recruited. High-resolution computed tomography (HRCT) scan provided evidence and patterns of RA-ILD. Serum sCD25 concentrations were measured by enzyme-linked immunosorbent assay (ELISA). Clinical and laboratory data were recorded and the association with sCD25 was also analysed. In the discovery cohort, 16 RA-related molecules including cytokines, chemokines and functional soluble cell surface proteins were investigated. The results showed that sCD25 was significantly higher in RA-ILD than in RA-no-ILD group (p=0.004). ROC analysis also showed RA-ILD was discriminated with RA-no-ILD by sCD25 (AUC=0.695, 95% CI=0.541-0.849). Logistics regression demonstrated that sCD25 was one of the risk factors of RA-ILD. This result was further confirmed in validation cohort (p<0.001). According to the cut-off value in the discovery cohort, the sensitivity and specificity of sCD25 in RA-ILD were 51.2%, 77.3%, respectively. Compared with RA-no-ILD, serum level of sCD25 was also higher in different HRCT patterns including UIP, NSIP and RA-ILA. The ROC curves revealed sCD25 as diagnostic marker in UIP, NSIP and RA-ILA (with AUCs of 0.730, 0.761, and 0. 694, respectively, p<0.05). The result indicated that sCD25 was a biomarker for RA-ILD subtypes. Although sCD25 was not correlated with HRCT scores, it was significantly higher in consolidation pattern by HRCT. sCD25 was significantly elevated in RA-ILD (including UIP, NSIP and RA-ILA) compared to RA-no-ILD and HCs, which supports their value as a potential biomarker in RA-ILD screening and assessment.

Updated severity and prognosis score of pulmonary alveolar proteinosis: A multi-center cohort study in China.

The high-resolution computed tomography (HRCT) score is an important component of the severity and prognosis score of pulmonary alveolar proteinosis (SPSP). However, the HRCT score in SPSP only considers the extent of opacity, which is insufficient. We retrospectively evaluated HRCT scores for 231 patients with autoimmune pulmonary alveolar proteinosis (APAP) from three centers of the China Alliance for Rare Diseases. The SPSPII was created based on the overall density and extent, incorporating the SPSP. The severity of APAP patients was assessed using disease severity scores (DSS), SPSP, and SPSPII to determine the strengths and weaknesses of the different assessment methods. We then prospectively applied the SPSPII to patients before treatment, and the curative effect was assessed after 3 months. The HRCT overall density and extent scores in our retrospective analysis were higher than the extent scores in all patients and every original extent score severity group, as well as higher related to arterial partial oxygen pressure (PaO2) than extent scores. The mild patients accounted for 61.9% based on DSS 1-2, 20.3% based on SPSP 1-3, and 20.8% based on SPSPII 1-3. Based on SPSP or SPSPII, the number of severe patients deteriorating was higher in the mild and moderate groups. When applied prospectively, arterial PaO2 differed between any two SPSPII severity groups. The alveolar-arterial gradient in PaO2 (P[A-a]O2), % predicted carbon monoxide diffusing capacity of the lung (DLCO), and HRCT score were higher in the severe group than in the mild and moderate groups. After diagnosis, mild patients received symptomatic treatment, moderate patients received pure whole lung lavage (WLL) or granulocyte-macrophage colony-stimulating factor (GM-CSF) therapy, and severe patients received WLL and GM-CSF therapy. Importantly, the SPSPII in mild and severe groups were lower than baseline after 3 months. The HRCT density and extent scores of patients with APAP were better than the extent score. The SPSPII score system based on smoking status, symptoms, PaO2, predicted DLCO, and overall HRCT score was better than DSS and SPSP for assessing the severity and efficacy and predicting the prognosis. ClinicalTrial.gov, identifier: NCT04516577.

Association of the Severity of Emphysema Based on HRCT Scoring System with Clinical Profile and Pulmonary Function Test Parameters: A Cross-sectional Study

Introduction: Chronic Obstructive Pulmonary Disease (COPD) is commonly observed in middle aged and elderly individuals. Dyspnoea, with or without expectoration, or isolated dyspnoea, is the primary respiratory symptom. Clinical signs, symptoms, and Pulmonary Function Tests (PFTs) are non specific. Chest radiography poorly correlates with disease severity and extent compared to clinical and functional impairment. High-Resolution Computed Tomography (HRCT) has been widely adopted to detect, characterise, and quantify emphysema. HRCT scoring is a useful radiological method for assessing emphysema severity in COPD patients and can provide prognostic information. Aim: To assess the severity of emphysema based on the HRCT scoring system and its association with clinical profile and PFTs. Materials and Methods: The present cross-sectional observational study was conducted in the Department of Radiodiagnosis at Atal Bihari Vajpayee Institute of Medical Sciences and Ram Manohar Lohia Hospital, New Delhi, India. It included 30 clinically diagnosed COPD patients referred for HRCT lung scans from November 1,2018, to March 31, 2020. HRCT assessment was performed at three levels: carina, 5 cm above carina, and 5 cm below carina. The severity of lung parenchyma was evaluated using the “Sakai Scoring Method.” The emphysema score was correlated with clinical profile (duration of illness, COPD severity, smoking, and pack-years smoked) and PFT parameters (FEV1, FEV1/FVC). Results: The mean age of the cases was 60±9.44 years. There was a strong positive linear correlation between the duration of illness (r=0.67, p=0.001) and COPD severity (r=0.452, p=0.02) with HRCT emphysema score. Significant correlation was found between HRCT emphysema score and pack-years smoked (r=0.558, p=0.004). The emphysema score showed an inverse correlation with FEV1 (r= -0.56, p=0.002) but no correlation with Forced Expiratory Volume 1 / Forced Vital Capacity (FEV1/FVC) (r= -0.16, p=0.430). Conclusion: The HRCT semi-quantitative scoring system is valuable for the initial assessment of disease severity and is significantly correlated with the PFT parameter FEV1.

The efficacy and safety of systemic corticosteroids as first line treatment for granulomatous lymphocytic interstitial lung disease

Granulomatous and lymphocytic interstitial lung disease (gl-ILD) is a major cause of morbidity and mortality among patients with common variable immunodeficiency. Corticosteroids are recommended as first-line treatment for gl-ILD, but evidence for their efficacy is lacking. This study analyzed the effect of high-dose corticosteroids (≥0.3 mg/kg prednisone equivalent) on gl-ILD, measured by high-resolution computed tomography (HRCT) scans, and pulmonary function test (PFT) results. Patients who had received high-dose corticosteroids but no other immunosuppressive therapy at the time (n= 56) and who underwent repeated HRCT scanning or PFT (n = 39) during the retrospective and/or prospective phase of the Study of Interstitial Lung Disease in Primary Antibody Deficiency (STILPAD) were included in the analysis. Patients without any immunosuppressive treatment were selected as controls (n= 23). HRCT scans were blinded, randomized, and scored using the Hartman score. Differences between the baseline and follow-up HRCT scans and PFT were analyzed. Treatment with high-dose corticosteroids significantly improved HRCT scores and forced vital capacity. Carbon monoxide diffusion capacity significantly improved in both groups. Of 18 patients, for whom extended follow-up data was available, 13 achieved a long-term, maintenance therapy independent remission. All patients with relapse were retreated with corticosteroids, but only one-fifth of them responded. Two opportunistic infections were found in the corticosteroid treatment group, while overall infection rate was similar between cohorts. Induction therapy with high-dose corticosteroids improved HRCT scans and PFT results of patients with gl-ILD and achieved long-term remission in 42% of patients. It was not associated with major side effects. Low-dose maintenance therapy provided no benefit and efficacy was poor in relapsing disease.

Airway clearance physiotherapy and health-related quality of life in cystic fibrosis.

ObjectiveAirway clearance physiotherapy is recommended in cystic fibrosis, but limited evidence exists to suggest how much treatment is enough. As a secondary analysis of a prior study investigating the safety, efficacy, and participants’ perceptions of a novel airway clearance technique, specific cough technique (SCT) compared to forced expiration technique (FET), we aimed to evaluate whether the intervention was associated with changes in health-related quality of life (HRQoL).MethodsWe conducted randomised, controlled individual trials with six adults (N-of-1 RCTs). Each trial included eight weeks of treatment, twice a week, using saline inhalation in horizontal positions, one with SCT and one with FET, in random order. Efficacy was measured by sputum wet weight (g) after each session. Perceived usefulness and preference were self-reported at the end of the study. Lung function was assessed at baseline and at the end of study. HRQoL was measured using the Cystic Fibrosis Questionnaire-Revised (CFQ-R) at baseline (week 1) and at completion of the study (week 8). Individual HRQoL scores (0–100) were coded and analysed using CFQ-R Software Program, version 2.0.ResultsPatient-reported outcomes were completed by all subjects. Individual CFQ-R-Respiratory Symptoms Scores (CFQ-R-RSS) showed a positive change, meeting the minimal important difference (MID) ≥ 4 points in five participants and a negative change in one individual. A strong correlation (r = 0.94 (p<0.01) was found between total sputum weight (g) and the positive changes in CFQ-R-RSS, and between changes in lung function and CFQ-R-RSS (r = 0.84 (p = 0.04).ConclusionThe airway clearance intervention was associated with clinically meaningful changes in patient-reported symptoms on the CFQ-R in the majority of the participants. This finding warrants further investigation regarding treatment, duration and frequency. A long-term study may reveal beneficial effects on other clinically meaningful endpoints, such as pulmonary exacerbations, high-resolution computed tomography scores and HRQoL.Trial registrationThe study was registered in ClinicalTrials.gov, under the number NCT0 1266473.

Factors associated with interstitial lung disease and the progressive fibrosing phenotype in rheumatoid arthritis–related interstitial lung disease

BackgroundThe risk factors for interstitial lung disease (ILD) in rheumatoid arthritis (RA) are inconsistent among previous studies. Furthermore, the factors associated with the emergence of the recently defined progressive fibrosing (PF) phenotype are unknown. Herein, we analyze the risk factors for ILD in RA. We also analyze the factors associated with a PF phenotype. MethodsWe collected the clinical and laboratory details of subjects with RA with (cases) or without (controls) ILD. Scoring of high-resolution computed tomography (HRCT) features of ILD was performed. We identified the subgroup that developed the PF phenotype during follow-up. We analyzed the factors associated with ILD using logistic regression (primary objective). We also compared the characteristics of ILD subjects with or without the PF phenotype (secondary objective). ResultsWe included 60 subjects (30 cases, 30 controls). Subjects with ILD had higher age, lower body mass index, longer duration of RA, and poorer lung function than the controls. Age (p = 0.007) and the duration of RA (p = 0.049) were the only significant predictors of ILD on univariate and multivariate analysis, respectively. Six (20%) subjects with RA-ILD developed a PF phenotype. These subjects were older, had greater frequency of honeycombing, and higher HRCT scores for honeycombing and aggregate fibrosis than those without the PF phenotype. Among subjects with honeycombing, 41.7% developed the PF phenotype. ConclusionsRA-ILD was associated with the duration of RA and age. Subjects with the PF phenotype were older and had higher honeycombing and fibrosis scores on HRCT chest.

Relationship between bronchopulmonary dysplasia phenotypes with high-resolution computed tomography score in early preterm infants.

ObjectiveTo assess the relationship between high-resolution computed tomography (HRCT) abnormalities and clinical phenotypes of bronchopulmonary dysplasia (BPD).MethodsA retrospective, single-center study was carried out at the Children’s Hospital of Fudan University between 2013 and 2020. Preterm infants born at ≤ 32 weeks’ gestation who were diagnosed with BPD and had HRCT between 40 and 50 weeks postmenstrual age (PMA)were included in the study. HRCT images from six pulmonary lobes were scored based on seven types of pulmonary lesions from two categories: hyperaeration lesions and parenchymal lesions. The hyperaeration score (HS) included scores of decreased attenuation, mosaic attenuation, and bulla/bleb, while the parenchymal score (PS) included those of linear lesion, consolidation, bronchial wall thickening, and bronchiectasis. All seven scores were summed up to create the total score (TS). One-way ANOVA testing or Kruskal-Wallis testing was adopted for the comparison of HRCT scores with BPD severity and clinical phenotypes. The correlation between HRCT scores and clinical phenotypes was evaluated by Spearman’s correlation analysis.ResultsA total of 81 cases were included in the study. Cases with more severe BPD had a higher TS (p = 0.01), HS (p = 0.02), PS (p = 0.02), mosaic attenuation score (p = 0.03), bulla/Bleb score (p = 0.03), and linear density score (p = 0.01). TS (r = 0.28), PS (r = 0.35), linear density (r = 0.34), and consolidation (r = 0.24) were correlated with pulmonary hypertension (PH). However, no HRCT score was significantly different between the patients with or without tracheobronchomalacia (TBM). BPD patients with a combination of lung parenchymal disease, PH, and TBM had the highest TS and HS.ConclusionHRCT scores correlated with BPD severity and PH in our study. HS might be a useful tool in the assessment of BPD severity while linear densities and consolidation might be helpful in predicting PH.

Incidence and Progression of Fibrotic Lung Disease in an At-Risk Cohort.

Rationale: Relatives of patients with familial interstitial pneumonia (FIP) are at increased risk for pulmonary fibrosis and develop preclinical pulmonary fibrosis (PrePF). Objectives: We defined the incidence and progression of new-onset PrePF and its relationship to survival among first-degree relatives of families with FIP. Methods: This is a cohort study of family members with FIP who were initially screened with a health questionnaire and chest high-resolution computed tomography (HRCT) scan, and approximately 4 years later, the evaluation was repeated. A total of 493 asymptomatic first-degree relatives of patients with FIP were evaluated at baseline, and 296 (60%) of the original subjects participated in the subsequent evaluation. Measurements and Main Results: The median interval between HRCTs was 3.9 years (interquartile range, 3.5-4.4 yr). A total of 252 subjects who agreed to repeat evaluation were originally determined not to have PrePF at baseline; 16 developed PrePF. A conservative estimate of the annual incidence of PrePF is 1,023 per 100,000 person-years (95% confidence interval, 511-1,831 per 100,000 person-years). Of 44 subjects with PrePF at baseline, 38.4% subjects had worsening dyspnea compared with 15.4% of those without PrePF (P = 0.002). Usual interstitial pneumonia by HRCT (P < 0.0002) and baseline quantitative fibrosis score (P < 0.001) are also associated with worsening dyspnea. PrePF at the initial screen is associated with decreased survival (P < 0.001). Conclusions: The incidence of PrePF in this at-risk population is at least 100-fold higher than that reported for sporadic idiopathic pulmonary fibrosis (IPF). Although PrePF and IPF represent distinct entities, our study demonstrates that PrePF, like IPF, is progressive and associated with decreased survival.

A Systematic Review and Metaanalysis of Predictors of Mortality in Idiopathic Inflammatory Myopathy-Associated Interstitial Lung Disease.

Idiopathic inflammatory myopathy (IIM)-associated interstitial lung disease (ILD) can range from rapidly progressive disease with high mortality to indolent disease with minimal morbidity. This systematic review and metaanalysis describe immunological, clinical, and radiographical predictors of mortality in IIM-ILD. MEDLINE and Embase database searches were completed on October 18, 2021, to identify articles providing survival data according to baseline characteristics in patients with concurrent IIM and ILD. Prognostic factors common to more than 5 papers were included in the metaanalysis using a random-effects model to report odds ratios (ORs) for binary variables and Hedges g for continuous variables. Risk of bias was assessed using the Newcastle-Ottawa Scale score and the Egger test for publication bias. From 4433 articles, 62 papers were suitable for inclusion; among these studies, 38 different variables were considered. The OR for risk of death regarding the presence of anti-melanoma differentiation-associated protein 5 (MDA5) antibodies was 6.20 (95% CI 3.58-10.71), and anti-tRNA synthetase antibodies were found to be protective (OR 0.24, 95% CI 0.14-0.41). Neither antinuclear antibodies, anti-52-kDa Ro antigen antibodies, nor SSA significantly altered mortality, nor was MDA5 titer predictive. Examples of prognostic factors that are significantly associated with mortality in this study include the following: age; male sex; acute/subacute onset; clinically amyopathic dermatomyositis; dyspnea; ulceration; fever; raised C-reactive protein, ferritin, lactate dehydrogenase, alveolar to arterial O2 (A-aO2) gradient, ground-glass opacity on high-resolution computed tomography (HRCT), and overall HRCT score; and reduced albumin, lymphocytes, ratio of partial pressure of oxygen in the arterial blood to fraction of inspired oxygen (PF ratio), percentage predicted transfer factor for carbon monoxide, and percentage predicted forced vital capacity. Baseline surfactant protein-D and Krebs von den Lungen-6 levels were not predictors of mortality. Many mortality risk factors were identified, though heterogeneity was high, with a low quality of evidence and a risk of publication bias. Studies regarding anti-MDA5 antibody-positive disease and and those from East Asia predominate, which could mask risk factors relevant to other IIM subgroups or populations.

The associations of radiological features of high-resolution computed tomography with the outcomes of transbronchial cryobiopsy in interstitial lung diseases: A cohort study.

BackgroundTransbronchial cryobiopsy (TBCB) is a critical procedure in the diagnosis of interstitial lung diseases (ILD). The associations between high-resolution computed tomography (HRCT) features and outcomes of TBCB were unknown.MethodsThis study was conducted as a single-center prospective cohort study between September 2018 and January 2020 (NCT04047667). HRCT was obtained before performing TBCB. The clinical and radiological characteristics, complications, pathological and multidisciplinary discussion (MDD) diagnoses were recorded. The relationships between HRCT features and outcomes of TBCB were analyzed.ResultsTBCB was performed on 216 ILD patients. The radiological features usually interstitial pneumonia (UIP) or probable UIP, indeterminate for UIP, ground-glass opacities (GGO) and cysts were found in 55 (25.5%), 38 (17.6%), 84 (38.9%) and 9 (4.2%) patients, respectively. And 118 (54.6%) patients had high HRCT score (involved lung proportion ≥50%) in the biopsied lobe. Multivariate analysis suggested radiological probable UIP pattern may be an independent risk factor for moderate bleeding (OR = 4.304; 95% CI: 1.383–13.393; P = 0.012), while GGO may be a protective factor from moderate bleeding (OR = 0.173, 95% CI: 0.043–0.687; P = 0.013). The pathological diagnostic yield in patients presenting cysts on HRCT was significantly lower than others (44.4 vs. 87.9%; P = 0.009). While performing TBCB in the lobe with high HRCT score increased pathological diagnostic yield (91.5 vs. 79.6%; P = 0.022). Neither pneumothorax nor MDD diagnostic yields were found to be associated with HRCT features.ConclusionsHRCT features were associated with moderate bleeding and pathological diagnosis. Pre-TBCB assessments of HRCT pattern and scores were helpful for bronchoscopists to make a better patient selection and procedure planning.

18F-FDG PET/CT and HRCT: a combined tool for risk stratification in idiopathic inflammatory myopathy-associated interstitial lung disease.

Rapidly progressive interstitial lung disease (RP-ILD) is a life-threatening form of idiopathic inflammatory myopathy (IIM)-associated interstitial lung disease (ILD). We aimed to assess the combination of 18F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET/CT) and high-resolution computed tomography (HRCT) for the quantification of IIM-ILD activity and risk stratification for RP-ILD. Patients with IIM and undergoing 18F-FDG PET/CT were included in this retrospective study. Pulmonary FDG uptake was assessed using the maximum standardized uptake value (SUVlung) and visual score (PET score). HRCT was evaluated using visual analysis (HRCT score). Multivariable logistic regression was used to identify risk factors for RP-ILD. Seventy-three patients with IIM (17 with RP-ILD, 38 with non-RP-ILD, and 18 without ILD) were included. SUVlung, PET score, and HRCT score were significantly higher in RP-ILD than in non-RP-ILD. Strong positive correlations were observed between SUVlung, PET score, and the HRCT parameters. The area under the curve (AUC) of the PET score to differentiate between RP-ILD and non-RP-ILD (AUC = 0.860) was higher than that of the SUVlung (AUC = 0.802) and HRCT scores (AUC = 0.806). We developed a risk score based on the number of positive risk factors (PET score > 18, HRCT score > 140, and positive anti-melanoma differentiation-associated gene 5 (MDA5) antibody) to differentiate between RP-ILD and non-RP-ILD (AUC = 0.955). Patients with higher risk scores had significantly worse prognoses. 18F-FDG PET/CT is useful for assessing disease activity in patients with IIM-ILD. The combination of PET score, HRCT score, and anti-MDA5 antibody can be used to identify patients at increased risk of RP-ILD and with poor prognoses.