High-resolution Computed Tomography Research Articles

Interstitial Lung Disease in Primary Sjögren's Syndrome: Risk factors for occurrence and radiographic progression.

The aim of this study is to investigate the characteristics of Primary Sjögren's syndrome (pSS)- interstitial lung disease (ILD) patients and compare them to those of pSS patients without ILD in the tertiary pSS-ILD cohort to evaluate potential risk factors for ILD occurrence and disease progression. Patients followed up who met the 2016 American College of Rheumatology-European League Against Rheumatism classification criteria for pSS were retrospectively analyzed. The patients were grouped as those with ILD and those without ILD according to medical records. High-resolution computed tomography (HRCT)/ thorax CT (TCT) results of all ILD patients were evaluated. Data on demographics, comorbidities, clinical characteristics and laboratory findings were collected. A total of 378 pSS patients, including 60 with ILD and 318 without ILD were detected to have at least one obtainable HRCT/TCT and were included in the study. In the cohort of pSS patients with at least one HRCT or TCT, the frequency of ILD was 15.8%. In the ILD group, the most common HRCT pattern was NSIP, and the most common findings were ground glass opacities, traction bronchiectasis, and honeycombing. Logistic regression analysis showed that male gender (OR:2.90), being diagnosed with pSS over the age of 50(OR:4,24), smoking history (OR:2.38), elevated LDH(OR:3.27), elevated ESR(OR:2.51) and lymphopenia (OR:5.12) were related with development of ILD while being diagnosed with ILD after the age of 60 (OR:8.5) was related with radiographic progression. The study results provided a large spectrum view for pSS-ILD and pointed out several risk factors for ILD occurrence and radiographic progression.

The lung in inborn errors of immunity.

The lungs are integral to immune defense, and inborn errors of immunity (IEI) often manifest as lung disease. Lung complications of IEI can involve the airways, alveolar spaces, interstitium, vasculature, and pleura. Accurate identification of these lung disease patterns requires a thorough clinical history, physical examination, and high-resolution computed tomography (HRCT), as lung imaging patterns guide further respiratory and immunological evaluations. Respiratory assessment may also include pulmonary function tests, bronchoscopy with bronchoalveolar lavage, and, in some cases, lung biopsy. Additionally, molecular diagnosis of underlying immune defects, typically through comprehensive clinical phenotyping, functional immune studies, and genetic testing, is crucial for informing patient management and guiding targeted therapies. Importantly, given the complexity of IEI, a multidisciplinary approach is necessary. Furthermore, ongoing research is required to refine therapies and improve outcomes for lung complications.

Low-dose high-resolution chest CT in adults with cystic fibrosis: intraindividual comparison between photon-counting and energy-integrating detector CT

BackgroundRegular disease monitoring with low-dose high-resolution (LD-HR) computed tomography (CT) scans is necessary for the clinical management of people with cystic fibrosis (pwCF). The aim of this study was to compare the image quality and radiation dose of LD-HR protocols between photon-counting CT (PCCT) and energy-integrating detector system CT (EID-CT) in pwCF.MethodsThis retrospective study included 23 pwCF undergoing LD-HR chest CT with PCCT who had previously undergone LD-HR chest CT with EID-CT. An intraindividual comparison of radiation dose and image quality was conducted. The study measured the dose-length product, volumetric CT dose index, effective dose and signal-to-noise ratio (SNR). Three blinded radiologists assessed the overall image quality, image sharpness, and image noise using a 5-point Likert scale ranging from 1 (deficient) to 5 (very good) for image quality and image sharpness and from 1 (very high) to 5 (very low) for image noise.ResultsPCCT used approximately 42% less radiation dose than EID-CT (median effective dose 0.54 versus 0.93 mSv, p < 0.001). PCCT was consistently rated higher than EID-CT for overall image quality and image sharpness. Additionally, image noise was lower with PCCT compared to EID-CT. The average SNR of the lung parenchyma was lower with PCCT compared to EID-CT (p < 0.001).ConclusionIn pwCF, LD-HR chest CT protocols using PCCT scans provided significantly better image quality and reduced radiation exposure compared to EID-CT.Relevance statementIn pwCF, regular follow-up could be performed through photon-counting CT instead of EID-CT, with substantial advantages in terms of both lower radiation exposure and increased image quality.Key PointsPhoton-counting CT (PCCT) and energy-integrating detector system CT (EID-CT) were compared in 23 people with cystic fibrosis (pwCF).Image quality was rated higher for PCCT than for EID-CT.PCCT used approximately 42% less radiation dose and offered superior image quality than EID-CT.Graphical

Predicting invasiveness of ground-glass nodules in lung adenocarcinoma: based on preoperative 18 F-fluorodeoxyglucose PET/computed tomography and high-resolution computed tomography.

This study was conducted to explore the differential diagnostic value of PET/computed tomography (PET/CT) combined with high-resolution computed tomography (HRCT) in predicting the invasiveness of ground-glass nodules (GGNs). This retrospective analysis included 67 patients (mean age 62.5 ± 8.4, including 45 females and 22 males) with GGNs who underwent preoperative 18 F-fluorodeoxyglucose ( 18 F-FDG) PET/CT and HRCT examinations between January 2018 and October 2022. Based on the postoperative pathological results of lung adenocarcinoma, the patients were classified into two groups: invasive adenocarcinoma (IAC) and non-IAC. Besides, the clinical and imaging information of these patients was collected. HRCT signs include the existence of air bronchial signals, vascular convergence, pleural indentation, lobulation, and spiculation. Moreover, the diameter of solid components (D Solid ), diameter of ground-glass nodules (D GGN ), and computed tomography values of ground-glass nodules (CT GGN ) were measured concurrently. Furthermore, the mean standardized uptake value, maximal standardized uptake value (SUVmax), metabolic tumor volume, and total lesion glycolysis were assessed during PET/CT. Associations between invasiveness and these factors were evaluated using univariate and multivariate analyses. The results of logistic regression analysis demonstrated that D GGN , D Solid , consolidation tumor ratio (CTR), CT GGN , and SUVmax were independent predictors in the IAC group. The combined diagnosis based on these five predictors revealed that area under the curve was 0.825. The D GGN , D Solid , CTR, CT GGN , and SUVmax in GGNs were independent predictors of IAC, and combining 18 F-FDG PET/CT metabolic parameters with HRCT may improve the predictive value of pathological classification in lung adenocarcinoma.

Chest wall muscle area, ventilatory efficiency and exercise capacity in systemic sclerosis.

To investigate the potential contribution of chest wall muscle area (CWMA) to the ventilatory efficiency and exercise capacity in patients with Systemic Sclerosis (SSc) without interstitial lung disease (ILD). Forty-four consecutive SSc patients [F = 37, median age 53.5years (IQR 43.5-58)] were examined using chest high-resolution computed tomography (HRCT), pulmonary function tests and cardiopulmonary exercise testing (CPET). The CWMA was evaluated at the level of the ninth thoracic vertebra on CT images by two independent evaluators blinded to the patient information. CPET parameters analyzed were maximum oxygen uptake (VO2 max) and VO2 at anaerobic threshold (VO2@AT); minute ventilation (VE); maximum tidal volume (VT). A statistically significant positive correlation was found between CWMA and maximum workload (r = 0.470, p < 0.01), VO2 max ml/min (r = 0.380, p < 0.01), VO2@AT (r = 0.343, p < 0.05), VE (r = 0.308, p < 0.05), VT (r = 0.410, p < 0.01) and VO2/heart rate (r = 0.399, p < 0.01). In multiple regression analysis, VO2 max (ml/min) was significantly associated with CWMA [β coefficient = 5.226 (95% CI 2.824, 7.628); p < 0.001], diffusing capacity for carbon monoxide (DLco) [β coefficient = 6.749 (95% CI 1.460, 12.039); p < 0.05] and body mass index (BMI) [β coefficient = 41.481 (95% CI 8.802, 74.161); p < 0.05]. In multiple regression analysis, maximum workload was significantly associated with CWMA [β coefficient = 0.490 (95% CI 0.289, 0.691); p < 0.001], DLco [β coefficient = 0.645 (95% CI 0.202, 1.088); p < 0.01] and BMI [β coefficient = 3.747 (95% CI 1.013, 6.842); p < 0.01]. In SSc-patients without ILD, CWMA represents an important variable in exercise capacity and can be evaluated by the mediastinal window available in the HRCT images required for lung disease staging.

Minimum intraoperative testing battery for cochlear implantation: the international practice trend.

In cochlear implantation (CI) surgery, there are a wide variety of intraoperative tests available. However, no clear guide exists on which tests must be performed as the minimum intraoperative testing battery. Toward this end, we studied the usage patterns, recommendations, and attitudes of practitioners toward intraoperative testing. This study is a multicentric international survey of tertiary referral CI centers. A survey was developed and administered to a group of CI practitioners (n = 34) including otologists, audiologists and biomedical engineers. Thirty six participants were invited to participate in this study based on a their scientific outputs to the literature on the intraoperative testing in CI field and based on their high load of CI surgeries. Thirty four, from 15 countries have accepted the invitation to participate. The participants were asked to indicate the usage trends, perceived value, influence on decision making and duration of each intraoperative test. They were also asked to indicate which tests they believe should be included in a minimum test battery for routine cases. Thirty-two (94%) experts provided responses. The most frequently recommended tests for a minimum battery were facial nerve monitoring, electrode impedance measurements, and measurements of electrically evoked compound action potentials (ECAPs). The perceived value and influence on surgical decision-making also varied, with high-resolution CT being rated the highest on both measures. Facial nerve monitoring, electrode impedance measurements, and ECAP measurements are currently the core tests of the intraoperative test battery for CI surgery.

Erosion regression in patients with rheumatoid arthritis after upadacitinib-a pilot study using high resolution peripheral quantitative computed tomography.

To evaluate whether inhibition of Janus kinases (JAK) 1 could lead to erosion repair on high-resolution peripheral quantitative computer tomography (HR-pQCT) in patients with active rheumatoid arthritis (RA). This was a prospective, non-randomized pilot study. We enrolled 20 adult patients with active RA with ≥1 bone erosion on HR-pQCT. They were given upadacitinib 15 mg once daily for 24 weeks. HR-pQCT of the metacarpophalangeal joint was performed at baseline and 24-week. The serum bone biomarkers level was evaluated before and after treatment. Twenty age-and-sex matched RA patients from another study treated with conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs) were included as active controls. Nineteen patients in the upadacitinib group completed the study procedures. After 24 weeks, despite similar improvement in disease activity, a reversed trend in the mean erosion volume change on HR-pQCT was observed comparing the upadacitinib and active control group (upadacitinib group: -0.23 ± 3.26mm3 vs control group: 1.32 ± 6.05mm3, p= 0.131). A greater proportion of erosions in the upadacitinib group demonstrated regression (27% vs 12%, p= 0.085). Using general estimating equation (GEE), the use of upadacitinib was significantly associated with erosion regression (OR: 3.61, 95% CI: 1.00-13.00, p= 0.049) after adjusting for the difference in disease duration. The serum levels of bone resorption markers reduced after upadacitinib treatment. No new safety signal was noted. Despite a similar improvement in RA disease activity after upadacitinib compared with csDMARDs, a differential regression of erosion on HR-pQCT was observed in patients received upadacitinib. The potential role of JAK1 inhibition in erosion repair should be investigated.

Intermittent fasting for systemic triglyceride metabolic reprogramming (IFAST): Design and methods of a prospective, randomized, controlled trial

BackgroundCaloric restriction prolongs lifespan in model organisms and improves metrics of aging-related diseases in humans, but daily compliance is challenging. Intermittent fasting improves metrics of lipid and glucose metabolism in the setting of weight loss but whether these metrics are improved independent of weight loss is not known. MethodsWe seek to address this gap with IFAST, a single-center, three-arm, prospective, randomized, controlled clinical trial. Eligible study participants are adults with no chronic medical conditions beyond prediabetes or overweight but who are at high risk for type 2 diabetes mellitus (T2D), defined as having a history of gestational diabetes or a first-degree relative with T2D. Participants will be randomized in a 1:2:2 schema to either a control group, a fasting group, or a fasting/weight maintenance group. The fasting groups will complete a 24-h fast one day per week for 12 weeks. The key mechanistic endpoint is change in triglyceride composition (defined by carbon content and degree of saturation) as measured by longitudinal metabolomics. The key safety endpoint is percent change from baseline in bone volume fraction (BV/TV; high-resolution peripheral quantitative CT) at the radius in the fasting group. Secondary endpoints include measures of insulin sensitivity (hyperinsulinemic-euglycemic clamp), clinical lipid profiling, systemic inflammation markers, hunger assessment, bone density, and bone microarchitecture with high-resolution peripheral quantitative CT. ConclusionIFAST will investigate intrinsic metabolic benefits of intermittent fasting beyond weight loss. Trial registrationClinicalTrials.gov ID NCT05722873.

Factors associated with interstitial lung disease in patients with rheumatoid arthritis

Aim: Interstitial lung disease (ILD) is a significant extra-articular manifestation of rheumatoid arthritis (RA). The prevalence and risk factors for ILD in RA exhibit considerable variation. This study aimed to determine the prevalence and factors associated with ILD in Thai patients with RA. Methods: This cross-sectional study enrolled 290 RA patients from the Siriraj Rheumatoid Arthritis Registry between March and December 2022. Patient characteristics, disease activity and functional status were documented. Chest radiography (CXR) was employed for ILD screening, and suspected cases were further evaluated using high-resolution computed tomography (HRCT) and pulmonary function tests. Two radiologists independently analyzed CXR and HRCT images, with any discrepancies resolved by a pulmonologist. Results: Among the 290 patients, the majority were female (89.7%) with mean age (SD) of 58.8 (11.5) years, and the median disease duration was 10 years (range 6–17 years). Patients exhibited low disease activity [mean Disease Activity Score 28-erythrocyte sedimentation rate score (SD) 2.69 (0.90)] and mild functional impairment [median Health Assessment Questionnaire score (range) 0.25 (0–0.63)]. Thirteen patients (4.5%) were diagnosed with ILD via HRCT (RA-ILD), with nonspecific interstitial pneumonia being the predominant ILD pattern (69.2%). Pulmonary function tests showed normal results in most patients, with only 15.5% presenting restrictive ventilatory defects. Age (P = 0.04), breathlessness (P &lt; 0.001), crackles (P &lt; 0.001), and functional impairment (P = 0.02) exhibited significant associations with RA-ILD. Conclusions: ILD is relatively infrequent in Thai patients with RA. However, older age, breathlessness, crackles, and functional impairment should prompt investigations for ILD in RA patients.

Clinicopathological and genetic analysis of interstitial disease-like pulmonary intravascular large B cell lymphoma

Objective: To investigate the clinicopathological features and genetic mutation status of pulmonary intravascular large B cell lymphoma. Methods: The clinicopathological data of eight patients diagnosed with pulmonary intravascular large B cell lymphoma, from April 2018 to May 2023, were retrospectively analyzed. The genetic profile of six patients was detected via next-generation sequencing (NGS) and followed up. Results: All patients included one male and seven females, with a median age of 64 years (ranging from 45 to 66 years). Respiratory symptoms were the most common (7 cases), B symptoms in two cases, hemophagocytic syndrome in two cases. Multiple diffuse ground-glass opacities in both lungs were observed based on the high-resolution chest CT scan. Six cases of mild to moderate ventilation or diffusion dysfunction were observed based on the pulmonary function tests. Moreover, two cases of hypoxemia and two cases with type Ⅰ respiratory failure were recorded. The serum lactate dehydrogenase level increased (7/8), β2-MG level increased (2/8), neuron-specific enolase level increased (7/8), total number of peripheral blood lymphocytes decreased (7/8), and clinical stages were all stage Ⅳ. The neoplastic lymphoid cells were lodged in the lumina of venules and capillaries of the alveolar septum; the tumor cells were large, with prominent nucleoli and frequent mitotic figures. The malignant cells were detected in the extravascular surrounding lung tissue in all cases. The tumor cells expressed mature B cell-associated antigens CD20 and CD79a, and the vascular endothelial markers CD31 and CD34 showed that the tumor cells were filled in the blood vessels, infiltrated blood vessel walls, and perivascular areas. One case was germinal center-type, seven cases were non-germinal center-type, two cases were double-expressing lymphoma, and all cases were EBER-negative. Furthermore, the top five genes with mutation frequencies detected by NGS were MYD88 (5/6), PIM1 (5/6), CD79B (4/6), TCF3 (4/6), and TP53 (3/6). Of the eight cases, seven patients received R-CHOP-based chemotherapy, six cases had complete remission after chemotherapy, one case died, and one case was lost to follow-up. Conclusions: Pulmonary vascular large B cell lymphoma is rare, which shares similar patterns with interstitial lung disease on imaging. Transbronchial lung biopsy is an effective method to confirm the diagnosis. Immunochemotherapy with BTK inhibitors can provide a survival advantage for patients in the future based on molecular typing.

The value analysis of high-resolution thin-layer CT in the identification of early lung adenocarcinoma: An observation study.

The aim of this study was to explore the clinical value of high-resolution thin-layer computed tomography (CT) for the identification of early lung adenocarcinoma. Ninety patients with early lung adenocarcinoma who were diagnosed and treated in our hospital were selected as study subjects and divided into noninvasive (NIG, n = 51) and invasive (IG, n = 39) groups according to their pathological findings. Both groups underwent high-resolution target scanning. Differences in lesion size, density, and distribution between the 2 groups were compared. Intergroup differences in the CT signs were examined. A receiver-operating characteristic curve was established to calculate the diagnostic efficacy of high-resolution, thin-layer CT for early lung adenocarcinoma infiltration. The maximum diameter and density of the tumors were significantly higher in the IG than in the NIG (P < .05). The proportions of CT signs of lobulation, spicule, and vessel convergence were higher in the IG patients compared to the NIG (P < .05). High-resolution thin-layer CT for the diagnosis of lung adenocarcinoma infiltration had an AUC of 0.6702 (P < .05), a diagnostic sensitivity of 64.10%, and a diagnostic specificity of 60.78%. High-resolution thin-layer CT had certain differential diagnostic efficacy for early lung adenocarcinoma, which clearly presents various CT signs of early lung adenocarcinoma lesions.

Novel deep-learning analysis for connective tissue disease -related interstitial lung disease extent assessment on CT: a preliminary cross-sectional study.

Physician's evaluation of interstitial lung disease (ILD) extension with high-resolution computed tomography (HRCT) has limitations such as lack of objectivity and reproducibility. This study aimed to investigate the utility of computer-based deep-learning analysis using QZIP-ILD® software (DL-QZIP) compared with conventional approaches in connective tissue disease (CTD) -related ILD. Patients with CTD-ILD visiting our Rheumatology Centre between December 2020 and April 2024 were recruited. Quantitative scores, including the percentage of lung involvement in ground-glass opacity (QGG), total fibrotic lesion (QFIB), and overall ILD extension encompassing both QGG and QFIB (QILD), calculated by DL-QZIP, were compared with semiquantitative visual method, employing intraclass correlation coefficients (ICC). We compared the capability of QILD scores to distinguish patients with forced vital capacity (FVC) % <70 in both methods determined by the area under the curve (AUC) by the receiver-operating characteristic curve analysis and DeLong's test. Eighty patients (median age, 66 years; 14 men) were included. Median QGG, QFIB, and QILD scores were 3.45%, 2.19%, and 5.35% using DL-QZIP, and 3.25%, 4.06%, and 8.48% using visual method, respectively. Correlations between DL-QZIP and visual method were 0.75 for QGG, 0.61 for QFIB, and 0.75 for QILD. The AUC of QILD scores for FVC% <70 was significantly higher with DL-QZIP (0.833) compared with visual method (0.660) (p < 0.01). QZIP-ILD® demonstrates superior capability in distinguishing patients with a radiological scenario correlated to severe physiological impairment, while showing relatively good correlations in quantifying the extent on HRCT compared with conventional method in CTD-ILD.

What is the Link between Lung Involvement and Anti-CCP Antibodies in Rheumatoid Arthritis: A Cross-sectional Study.

In Rheumatoid Arthritis (RA), pulmonary involvement is one of the most frequent extra-articular manifestations. Several studies have demonstrated an association between RA-related lung disease and the positivity of anti-cyclic citrullinated peptide (anti-CCP) antibodies. Our aim is to describe the frequency of pulmonary involvement in the RA population and investigate the association between anti-CCP antibodies and diverse lung compartment involvement in RA patients. An observational retrospective cross-sectional study was conducted, during which data were collected from the medical records of the patients with RA who had been tested for anti-CCP antibodies and had thoracic high resolution computerized tomography (HRCT) evaluation from January 2011 to March 2022. The univariate and multivariate analyses using logistic regression models was performed to calculate odds ratios (ORs) with 95% CIs. A total of 390 patients with RA were included, the mean age of patients was 58.99 ± 12.44 years, with a predominance of females (85.9%). Two hundred and fifty-two (64.6%) patients were positive for anti-CCP antibodies. The frequency of RA-related lung diseases was 14.4% (n=56). The different manifestations observed in the thoracic HRCT included Nodules (67.9%), Interstitial lung disease (ILD) (28.6%), bronchiectasis (25%), fibrosis (21.4%), obliterative bronchiolitis (7.1%), and pleuritis (1.8%). In univariate and multivariate analysis, pulmonary involvement was associated with positive anti-CCP antibodies with an odds ratio (OR) of 5.25 (95% CI: 2.17-12.70, p < 0.0001). The study demonstrated a positive association between anti-CCP antibodies and pulmonary involvement in RA and highlighted the importance of tight monitoring in RA patients with positive anti-CCP for pulmonary complications.

Effectiveness of Amikacin liposome inhalation suspension for refractory Mycobacterium avium complex pulmonary disease at 6 months post initiation

BackgroundAmikacin liposome inhalation suspension (ALIS) improved sputum culture conversion rate at 6 months for patients with refractory Mycobacterium avium complex pulmonary disease (MAC-PD) in an international phase 3 trial. Patient characteristics and chest high-resolution CT (HRCT) findings associated with ALIS effectiveness are poorly documented.ObjectiveWe aimed to clarify ALIS effectiveness for refractory MAC-PD at 6 months, elucidating associated patient characteristics and chest CT findings.MethodsWe reviewed medical records of 12 patients with refractory MAC-PD for whom ALIS treatment was initiated at Toho University Omori Medical Center from November 2021 through September 2022. All patients demonstrated treatment persistence for at least 3 months. They were divided into culture conversion and non-conversion groups using sputum culture conversion status after 6-month ALIS treatment initiation. Clinical and radiological characteristics were compared.ResultsSeven of the 12 patients (58.3%) achieved sputum culture conversion within 6 months. The culture conversion group had shorter pre-ALIS initiation treatment duration [21 months (16–25) vs. 62 months (32–69); p = 0.045]; lower cavitary lesion incidence on HRCT (28.6% vs. 100%; p = 0.028); and fewer clarithromycin (CLA)-resistant strains [0/7 (0%) vs. 3/5 (60%); p = 0.045]. Chest HRCT findings improved in 4 of 7 (57.1%) and 1 of 5 (20%) patients in the culture conversion and non-conversion groups, respectively.ConclusionALIS facilitated sputum culture conversion within 6 months in 58.3% of patients with refractory MAC-PD. Sputum culture conversion was significantly more frequent for CLA-susceptible strains and patients with fewer cavitary lesions. Improved CT findings after ALIS did not always correspond to sputum culture conversion.

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.

Pulmonary alveolar proteinosis: presentation, diagnostic challenges, and management.

Pulmonary alveolar proteinosis is a rare diffuse lung disease; diagnosis and treatment of which is often delayed. We present the case study of a 43-year-old male with a six-month history of worsening breathlessness and non-productive cough referred for specialist respiratory input. Rapid investigations, including high-resolution computed tomography (HRCT) and bronchoalveolar lavage, confirmed the diagnosis of pulmonary alveolar proteinosis. Treatment with whole lung lavage significantly improved pulmonary function and quality of life. We discuss the diagnosis and management of this condition and highlight the importance of early recognition and multidisciplinary teamwork in managing pulmonary alveolar proteinosis.

Clinical manifestations, treatment and prognosis of juvenile idiopathic arthritis with pulmonary involvement in China: a single centre study.

In recent years, the distinct clinical presentations and elevated mortality rates of various subtypes of juvenile idiopathic arthritis (JIA) with pulmonary involvement have garnered significant attention. This study aimed to elucidate the clinical characteristics of pulmonary involvement in patients with JIA to improve clinicians' knowledge. This single-centre retrospective study analysed the baseline data, treatment options, follow-up of sixty patients of JIA with pulmonary involvement in China. Patients with interstitial lung disease (ILD) were further classified in accordance with the 2013 American Thoracic Society/European Respiratory Society International multidisciplinary consensus on idiopathic interstitial pneumonia. Sixty patients (5.03%) with JIA were complicated with pulmonary involvement. The highest subtype was systemic JIA (sJIA, 63.3%), followed by rheumatoid factor (RF)-positive polyarthritis (pJIA, 25.0%). The incidence of macrophage activation syndrome (MAS) was 21.6%. The most common diagnosis was ILD (90%). Respiratory symptoms/signs were initially experienced by 61.7% of the patients, and respiratory support was required by 21.7%. High-resolution CT classification of sJIA revealed non-specific interstitial pneumonia (NSIP) and organising pneumonia. High-resolution CT classification of pJIA was NSIP and usually interstitial pneumonia (UIP). Patients were treated with NSAIDs, along with glucocorticoids, DMARDs, and biological agents. The survival rates after 1 and 5 years were approximately 93.3% and 90.0%, respectively. Patients with JIA with pulmonary involvement present with early onset, high mortality rate. JIA patients should undergo physical examination thoroughly and high-resolution CT scans, lung function tests for evaluating and monitoring the occurrence and development of pulmonary involvement in early stages to improve prognosis.

Selection of inner ear fenestration strategy and surgical effect of patients with oval window atresia accompanied by facial nerve aberration

Objective: To summarize the clinical features and postoperative efficacy of patients with oval window atresia accompanied by facial nerve aberration. Methods: The clinical data of patients with congenital middle ear malformation with facial nerve aberration admitted to our hospital from January 2015 to March 2023 were retrospectively analyzed. There were 97 cases (133 ears) in total. Among them, 39 patients (44 ears) had complete follow-up data, including 27 male patients and 12 females, aged 7-48 years old, with an average age of 17.8 years old. Of these, 14 cases (16 ears) were patients combined with facial nerve aberration, and 25 cases (28 ears) were without facial nerve aberration. The results of imaging examination, pure-tone audiometry, selection of surgical strategy, intraoperative findings and postoperative hearing improvement were summarized and analyzed. The malformations of malleus, incus, stapes, oval window and facial nerve were recorded. Prism 9 software was used to statistically analyze the mean bone conductance and air-bone gap of patients before and after surgery. Results: All the 14 patients (16 ears) with middle ear malformation accompanied by facial nerve aberration and oval window atresia showed poor hearing and no facial palsy since childhood. High resolution CT (HRCT) examination of temporal bone, pure tone audiometry and Gelle test were performed before surgery. The malformations of malleus, incus, stapes, oval window and facial nerve were recorded. Preoperative high-resolution CT (HRCT) examination of temporal bone found 12 ears with 4 or more deformities, accounting for 75.00%, in the group of patients with facial nerve malformation. The preoperative average bone conductive threshold was (15.3±10.4) dB and the average air-bone gap was (46.3±10.6) dB in pure-tone audiometry (0.5, 1, 2, 4kHz). According to the different degrees of facial nerve and ossicle malformation, we performed three different hearing reconstruction strategies for the 14 patients (16 ears) with facial nerve aberration and oval window atresia, including 7 ears of incus bypass artificial stape implantation, 7 ears of Malleostapedotomy (MS) and 2 ears of Malleus-cochlear-prothesis (MCP). After 3 months to 18 months of follow-up, all patients showed no facial paralysis. The postoperative mean bone conductive threshold was (15.7±7.9) dB and air-bone gap was (19.8±8.5) dB. There were significant differences in mean air-bone gap before and after operation (t=7.766, P<0.05), and there was no significant difference between the mean bone conductive threshold before and after surgery (t=0.225, P=0.824). There was no significant difference of mean reduction of air-bone gap between patients with and without facial nerve aberration (t=1.412, P=0.165). There was no significant difference between the three hearing reconstruction strategies. There was no significant displacement of the Piston examined by U-HRCT. Conclusion: For patients of middle ear malformation whose facial nerve cover the oval window partially, incus bypass artificial stape implantation or Malleostapedotomy (MS) can be selected according to the specific condition of auditory ossis malformation, and for patients whose facial nerve completely covers the oval window area, Malleus-cochlear-prothesis (MCP) can be selected. Three types of stapes surgery are safe and reliable for patients with oval window atresia accompanied by facial nerve aberration. There was no significant difference in efficacy between them. Preoperative HRCT assessment of middle ear malformation is effective. There is no significant difference of surgical effect with or without facial nerve aberration. The U-HRCT can be used to evaluate the middle ear malformation before surgery and the Piston implantation status after surgery. Due to the risks of surgery, those who do not want to undergo surgery can choose artificial hearing AIDS, such as hearing aid, vibrating soundbridge, bone bridge or bone-anchored hearing aid.

Quantitative CT analysis of idiopathic pulmonary fibrosis and correlation with lung function study

BackgroundIdiopathic Pulmonary Fibrosis (IPF) is a progressive fibrotic lung disease. However, the field of quantitative CT scan analysis in conjunction with pulmonary function test for IPF patients remains relatively understudied. In this study, we evaluated the diagnostic value of features derived high-resolution computed tomography (HRCT) for patients with IPF and correlated them with pulmonary function tests.MethodsWe retrospectively analyzed the chest HRCT images and pulmonary function test results of 52 patients with IPF during the same period (1 week) and selected 52 healthy individuals, matched for sex, age, and body mass index (BMI) and with normal chest HRCT as controls. HRCT scans were performed using a Philips 256-row Brilliance iCT scanner with standardized parameters. Lung function tests were performed using a Jaeger volumetric tracer for forced vital capacity (FVC), total lung capacity (TLC), forced expiratory volume in first second (FEV1), FEV1/FVC, carbon monoxide diffusing capacity (DLCO), and maximum ventilation volume (MVV) metrics. CT quantitative analysis, including tissue segmentation and threshold-based quantification of lung abnormalities, was performed using 3D-Slicer software to calculate the percentage of normal lung areas (NL%), percentage of ground-glass opacity areas (GGO%), percentage of fibrotic area (F%) and abnormal lesion area percentage (AA%). Semi-quantitative analyses were performed by two experienced radiologists to assess disease progression. The aortic-to-sternal distance (ASD) was measured on axial images as a standardized parameter. Spearman or Pearson correlation analysis and multivariate stepwise linear regression were used to analyze the relationship between the data in each group, and the ROC curve was used to determine the optimal quantitative CT metrics for identifying IPF and controls.ResultsROC curve analysis showed that F% distinguished the IPF patient group from the control group with the largest area under the curve (AUC) of 0.962 (95% confidence interval: 0.85–0.96). Additionally, with F% = 4.05% as the threshold, the Youden’s J statistic was 0.827, with a sensitivity of 92.3% and a specificity of 90.4%. The ASD was significantly lower in the late stage of progression than in the early stage (t = 5.691, P < 0.001), with a mean reduction of 2.45% per month. Quantitative CT indices correlated with all pulmonary function parameters except FEV1/FVC, with the highest correlation coefficients observed for F% and TLC%, FEV1%, FVC%, MVV% (r = − 0.571, − 0.520, − 0.521, − 0.555, respectively, all P-values < 0.001), and GGO% was significantly correlated with DLCO% (r = − 0.600, P < 0.001). Multiple stepwise linear regression analysis showed that F% was the best predictor of TLC%, FEV1%, FVC%, and MVV% (R2 = 0.301, 0.301, 0.300, and 0.302, respectively, all P-values < 0.001), and GGO% was the best predictor of DLCO% (R2 = 0.360, P < 0.001).ConclusionsQuantitative CT analysis can be used to diagnose IPF and assess lung function impairment. A decrease in the ASD may indicate disease progression.

Associations between 6-minute walk distance and physiologic measures and clinical outcomes in myositis-associated interstitial lung disease.

The 6-min walk test (6MWT) is a simple test widely used to assess sub-maximal exercise capacity in chronic respiratory diseases. We explored the relationship of 6-min walk distance (6MWD) with measurements of physiological, clinical, radiographic measures in patients with myositis-associated interstitial lung disease (MA-ILD). We analyzed data from the Abatacept in Myositis Associated Interstitial lung disease (Attack My-ILD) study, a 48-week multicentre randomized trial of patients with anti-synthetase antibodies and active MA-ILD. 6MWD, forced vital capacity (FVC), diffusing capacity (DLCO), high resolution CT, and various physician/patient reported outcome measures were obtained during the trial. Spearman's correlations and repeated-measures analysis with linear mixed-effects models were used to estimate the associations between 6MWD and various physiologic, clinical and radiographic parameters both cross-sectionally and longitudinally. Twenty participants with a median age of 57, 55% male and 85% white were analyzed. Baseline 6MWD did not associate with baseline PFTs. Repeated-measures analysis showed 6MWD over time associated with FVC over time, but not with DLCO. 6MWD over time also correlated with UCSD dyspnea score, Borg scores, as well as global disease activity and muscle strength over time. Emotional role functioning, vitality, general health and physical functioning scores by short form 36 also correlated with 6MWD over time. : Exploratory work in a small cohort of MA-ILD demonstrated 6MWD over time associated with parallel changes in FVC and patient reported outcomes of dyspnea, but not with DLCO. Larger studies are needed to validate the reliability, responsiveness and utility of the 6MWT in MA-ILD. ClinicalTrials.gov, NCT03215927.