Lung imaging patterns in connective tissue disease-associated interstitial lung disease impact prognosis and immunosuppression response.

OBJECTIVES: Interstitial lung disease (ILD) in connective tissue diseases (CTD) have highly variable morphology. We aimed to identify imaging features and their impact on ILD progression, mortality and immunosuppression response. METHODS: Patients with CTD-ILD had high-resolution chest computed tomography (HRCT) reviewed by expert radiologists blinded to clinical data for overall imaging pattern (usual interstitial pneumonia [UIP]; non-specific interstitial pneumonia [NSIP]; organizing pneumonia [OP]; fibrotic hypersensitivity pneumonitis [fHP]; and other). Transplant-free survival and change in percent-predicted forced vital capacity (FVC) were compared using Cox and linear mixed effects models adjusted for age, sex, smoking, and baseline FVC. FVC decline after immunosuppression was compared with pre-treatment. RESULTS: Of 645 CTD-ILD patients, the frequent CTDs were systemic sclerosis (n = 215), rheumatoid arthritis (n = 127), and inflammatory myopathies (n = 100). NSIP was the most common pattern (54%), followed by UIP (20%), fHP (9%), and OP (5%). Compared with UIP, FVC decline was slower for NSIP (1.1%/year, 95%CI 0.2, 1.9) and OP (3.5%/year, 95%CI 2.0, 4.9), and mortality was lower for NSIP (HR 0.65, 95%CI 0.45, 0.93) and OP (HR 0.18, 95%CI 0.05, 0.57), but higher in fHP (HR 1.58, 95%CI 1.01, 2.40). The extent of fibrosis also predicted FVC decline and mortality. After immunosuppression, FVC decline was slower compared with pre-treatment in NSIP (by 2.1%/year, 95%CI 1.4, 2.8), with no change for UIP or fHP. CONCLUSION: Multiple radiologic patterns are possible in CTD-ILD, including a fHP pattern. NSIP and OP were associated with better outcomes and response to immunosuppression, while fHP had worse survival compared with UIP.

Integration and Application of Radiologic Patterns From Clinical Practice Guidelines on Idiopathic Pulmonary Fibrosis and Fibrotic Hypersensitivity Pneumonitis.

BACKGROUND: Clinical practice guidelines separately describe radiologic patterns of usual interstitial pneumonia (UIP) and fibrotic hypersensitivity pneumonitis (fHP), without direction on whether or how to apply these approaches concurrently within a single patient. RESEARCH QUESTION: How can we integrate guideline-defined radiologic patterns to diagnose interstitial lung disease (ILD) and what are the pitfalls associated with described patterns that require reassessment in future guidelines? STUDY DESIGN AND METHODS: Patients from the Canadian Registry for Pulmonary Fibrosis underwent detailed reevaluation in standardized multidisciplinary discussion. CT scan features were quantified by chest radiologists masked to clinical data, and guideline-defined patterns were assigned. Clinical data then were provided to the radiologist and an ILD clinician, who jointly determined the leading diagnosis. RESULTS: Clinical-radiologic diagnosis in 1,593 patients was idiopathic pulmonary fibrosis (IPF) in 26%, fHP in 12%, connective tissue disease-associated ILD (CTD-ILD) in 34%, idiopathic pneumonia with autoimmune features in 12%, and unclassifiable ILD in 10%. Typical and probable UIP patterns corresponded to a diagnosis of IPF in 66% and 57% of patients, respectively. Typical fHP pattern corresponded to an fHP clinical diagnosis in 65% of patients, whereas compatible fHP was nonspecific and associated with CTD-ILD or IPAF in 48% of patients. No pattern ruled out CTD-ILD. Gas trapping affecting > 5% of lung parenchyma on expiratory imaging was an important feature broadly separating compatible and typical fHP from other patterns (sensitivity, 0.77; specificity, 0.91). INTERPRETATION: An integrated approach to guideline-defined UIP and fHP patterns is feasible and supports > 5% gas trapping as an important branch point. Typical or probable UIP and typical fHP patterns have moderate predictive values for a corresponding diagnosis of IPF and fHP, although occasionally confounded by CTD-ILD; compatible fHP is nonspecific.

Interpretable machine learning for automated left ventricular scar quantification in hypertrophic cardiomyopathy patients.

Scar quantification on cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) images is important in risk stratifying patients with hypertrophic cardiomyopathy (HCM) due to the importance of scar burden in predicting clinical outcomes. We aimed to develop a machine learning (ML) model that contours left ventricular (LV) endo- and epicardial borders and quantifies CMR LGE images from HCM patients.We retrospectively studied 2557 unprocessed images from 307 HCM patients followed at the University Health Network (Canada) and Tufts Medical Center (USA). LGE images were manually segmented by two experts using two different software packages. Using 6SD LGE intensity cutoff as the gold standard, a 2-dimensional convolutional neural network (CNN) was trained on 80% and tested on the remaining 20% of the data. Model performance was evaluated using the Dice Similarity Coefficient (DSC), Bland-Altman, and Pearson's correlation. The 6SD model DSC scores were good to excellent at 0.91 ± 0.04, 0.83 ± 0.03, and 0.64 ± 0.09 for the LV endocardium, epicardium, and scar segmentation, respectively. The bias and limits of agreement for the percentage of LGE to LV mass were low (-0.53 ± 2.71%), and correlation high (r = 0.92). This fully automated interpretable ML algorithm allows rapid and accurate scar quantification from CMR LGE images. This program does not require manual image pre-processing, and was trained with multiple experts and software, increasing its generalizability.

Clinical Impact of Cardiac MRI T1 and T2 Parametric Mapping in Patients with Suspected Cardiomyopathy.

Background There are limited data on the incremental value of parametric mapping compared with core cardiac MRI protocols for suspected cardiomyopathy in routine clinical practice. Purpose To evaluate the impact of cardiac MRI T1 and T2 mapping in routine clinical practice with respect to diagnostic accuracy, reader diagnostic confidence, and downstream cardiac imaging utilization. Materials and Methods In this retrospective single-center study, consecutive clinical cardiac MRI scans obtained with and without T1 and T2 mapping for evaluation of suspected cardiomyopathy between January 2017 and October 2019 were evaluated. Diagnostic accuracy and reader diagnostic confidence were evaluated in a random subset. Downstream cardiac imaging utilization was analyzed in patients with a minimum of 1 year of clinical follow-up ending before January 2020. Results A total of 1876 patients (mean age, 51 years ± 17 [SD]; 1113 men) were evaluated. Of these, 751 (40%) underwent cardiac MRI with the core protocol and 1125 (60%) with the core protocol plus T1 and T2 mapping. In the mapping group, T1 and T2 were high in 280 (25%) and 47 patients (4%), respectively. In the subset evaluated for diagnostic utility (n = 450), the addition of T1 and T2 maps to the core protocol resulted in an improvement in reader diagnostic confidence in 174 patients (39%). Diagnostic sensitivity was higher with the core protocol plus mapping compared with the core protocol alone for myocarditis (89% [31 of 35 patients] vs 69% [24 of 35]; P = .008), Fabry disease (93% [13 of 14 patients] vs 50% [seven of 14]; P = .01), and amyloidosis (100% [16 of 16 patients] vs 63% [10 of 16]; P = .01). In the subset evaluated for downstream imaging utilization (n = 903), 47% of patients with mapping had at least one subsequent cardiac imaging test compared with 55% of patients without mapping (P = .01). Conclusion In patients with suspected cardiomyopathy, cardiac MRI with T1 and T2 mapping had high diagnostic utility and was associated with lower downstream cardiac imaging utilization. © RSNA, 2022 Online supplemental material is available for this article. See also the editorial by Jerosch-Herold and Coelho-Filho in this issue.

The Impact of Slice Thickness on Diagnostic Accuracy in Digital Breast Tomosynthesis.

Purpose: To evaluate the effect of slice thickness on diagnostic accuracy in Digital Breast Tomosynthesis (DBT). Method: Two readers retrospectively interpreted 150 DBT (125 normal and 25 pathology-proven cancer) cases scanned between October 2017-November 2020. The DBT studies were randomised and reviewed independently by the two readers. DBT studies were reviewed using a standard protocol (1 mm slices, no overlap and synthetic 2D-mammography (SM)) and an experimental protocol (10 mm slabs, 5 mm overlap and SM). Any abnormality and BIRADS scores were recorded by each reader. Sensitivity, specificity, interobserver and intraobserver agreement were calculated (Cohen's Kappa κ). For diagnostic accuracy, the reference standard was histopathology or a normal mammogram at 2 years. Results: The sensitivity and specificity for reader 1 and 2 for cancer detection was reader 1 (97% and 79% for the standard protocol, 97% and 76% for the experimental protocol) and reader 2 (97% and 74% for both protocols). Reader 1 had 97.6% intraobserver agreement (κ .95) and reader 2 had 96.4% intraobserver agreement (κ .92) when assessing the standard and experimental protocols. There was 90.5% agreement between the readers for the standard protocol (κ .80). There was 90.9% agreement between the readers for the experimental protocol (κ .81). Of the 25 DBT studies with pathology-proven cancer, one cancer was missed by both readers using both protocols. Conclusion: The diagnostic accuracy was similar between the standard and experimental DBT protocols, demonstrating excellent interobserver and intraobserver agreement. This suggests 10-mm thick slabs can potentially replace 1-mm thin slices in the interpretation of DBT.

An unusual case of nodular pulmonary amyloidosis.

Nodular pulmonary amyloidosis is a rare and localized manifestation of amyloid deposition in the lungs. This rare entity, though asymptomatic, is often misdiagnosed on imaging alone, due to its resemblance to metastatic pulmonary nodules. This report highlights the significance of histologic confirmation before treatment, as a preventive measure against overtreatment.

Type 2 bridging bronchus with left pulmonary artery sling.

Bridging bronchi are the rarest of the major airway anomalies reported in the literature. In this brief report, we present a case of a symptomatic adult male patient presenting with a type 2 bridging bronchus associated with left pulmonary artery sling.

Clinical Significance of Papillary Muscles on Left Ventricular Mass Quantification Using Cardiac Magnetic Resonance Imaging: Reproducibility and Prognostic Value in Fabry Disease.

PURPOSE: Accurate and reproducible assessment of left ventricular mass (LVM) is important in Fabry disease. However, it is unclear whether papillary muscles should be included in LVM assessed by cardiac magnetic resonance imaging (MRI). The purpose of this study was to evaluate the reproducibility and predictive value of LVM in patients with Fabry disease using different analysis approaches. MATERIALS AND METHODS: A total of 92 patients (44±15 y, 61 women) with confirmed Fabry disease who had undergone cardiac MRI at a single tertiary referral hospital were included in this retrospective study. LVM was assessed at end-diastole using 2 analysis approaches, including and excluding papillary muscles. Adverse cardiac events were assessed as a composite end point, defined as ventricular tachycardia, bradycardia requiring device implantation, severe heart failure, and cardiac death. Statistical analysis included Cox proportional hazard models, Akaike information criterion, intraclass correlation coefficients, and Bland-Altman analysis. RESULTS: Left ventricular end-diastolic volume, end-systolic volume, ejection fraction, and LVM all differed significantly between analysis approaches. LVM was significantly higher when papillary muscles were included versus excluded (157±71 vs. 141±62 g, P<0.001). Mean papillary mass was 16±11 g, accounting for 10%±3% of total LVM. LVM with pap illary muscles excluded had slightly better predictive value for the composite end point compared with LVM with papillary muscles included based on the model goodness-of-fit (Akaike information criterion 140 vs. 142). Interobserver agreement was slightly better for LVM with papillary muscles excluded compared with included (intraclass correlation coefficient 0.993 [95% confidence interval: 0.985, 0.996] vs. 0.989 [95% confidence interval: 0.975, 0.995]) with less bias and narrower limits of agreement. CONCLUSIONS: Inclusion or exclusion of papillary muscles has a significant effect on LVM quantified by cardiac MRI, and therefore, a standardized analysis approach should be used for follow-up. Exclusion of papillary muscles from LVM is a reasonable approach in patients with Fabry disease given slightly better predictive value and reproducibility.