Panlobular emphysema is associated with COPD disease severity: A study of emphysema subtype by computed tomography.

BACKGROUND: Computed tomography has the potential to inform COPD prognosis. We sought to determine associations of emphysema phenotype with clinical parameters including lung function, inflammatory markers, and quality of life. METHODS: Participants of this single-center observational cohort (n = 83) were 40-80 years old, had ≥10 pack-year smoking, and a diagnosis of COPD confirmed by spirometry. All participants had available historic chest CT scans which were systematically reviewed by a single expert radiologist and scored for emphysema subtype, extent, and distribution. Associations between radiographic findings and clinical parameters were determined. RESULTS: Median age of participants was 72 years, median smoking 40 pack-years, and median FEV1 59% predicted. 84% of the participants had radiographic emphysema. Of those, 26% had panlobular emphysema (PLE), 68% centrilobular emphysema (CLE), and 6% paraseptal emphysema (PSE). As compared to the participants with no radiographic emphysema, the presence of PLE-dominant emphysema was associated with a lower BMI (P = 0.012) and greater extent of emphysema (P = 0.014). After adjusting for age, sex, and pack-years smoking history, PLE was associated with greater airflow obstruction by FEV1% (48% vs 71%, P = 0.005), greater symptom burden by CAT score (18 vs 9, P = 0.015), worse quality of life by SGRQ score (43 vs 22, P = 0.025), and more systemic inflammation by erythrocyte sedimentation rate (P = 0.001). CLE- or PSE-dominant emphysema were not similarly associated with clinical features or symptom burden. CONCLUSIONS: The presence of PLE-dominant emphysema was associated with greater extent of emphysema, greater airflow obstruction, increased respiratory symptoms, worse quality of life, and systemic inflammation. Further investigation is indicated to explore the pathogenesis of the PLE phenotype and the prognostic and treatment implications of PLE.

A System-Wide Population Health Value Approach to Reduce Hospitalization Among Chronic Kidney Disease Patients: an Observational Study.

BACKGROUND: Chronic kidney disease (CKD) is a leading cause of healthcare morbidity, utilization, and expenditures nationally, and caring for late-stage CKD populations is complex. Improving health system efficiency could mitigate these outcomes and, in the COVID-19 era, reduce risks of viral exposure. OBJECTIVE: As part of a system-wide transformation to improve healthcare value among populations with high healthcare utilization and morbidity, UCLA Health evaluated a new patient-centered approach that we hypothesized would reduce inpatient utilization for CKD patients. DESIGN: For 18 months in 2015-2016 and 12 months in 2017, we conducted an interrupted time series regression analysis to evaluate the intervention's impact on inpatient utilization. We used internal electronic health records and claims data across six payers. PARTICIPANTS: A total of 1442 stage 4-5 CKD patients at a large academic medical center. INTERVENTION: Between October and December 2016, the organization implemented a Population Health Value CKD intervention for the CKD stages 4-5 population. A multispecialty leadership team risk stratified the population and identified improvement opportunities, redesigned multispecialty care coordination pathways across settings, and developed greater ambulatory infrastructure to support care needs. MAIN MEASURES: Outcomes included utilization of hospitalizations, emergency department (ED) visits, inpatient bed days, and 30-day all-cause readmissions. KEY RESULTS: During the 12 months following intervention implementation, the monthly estimated rate of decline for hospitalizations was 5.4% (95% CI: 3.4-7.4%), which was 3.4 percentage points faster than the 18-month pre-intervention decline of 2.0% (95% CI: 1.0-2.2%) per month (p = 0.004). Medicare CKD patients' monthly ED visit rate of decline was 3.0% (95% CI: 1.2-4.8%) after intervention, which was 2.6 percentage points faster than the pre-intervention decline of 0.4% (95% CI: - 0.8 to 1.6%) per month (p = 0.02). CONCLUSIONS: By creating care pathways that link primary and specialty care teams across settings with increased ambulatory infrastructure, healthcare systems have potential to reduce inpatient healthcare utilization.

Brief report: effect of ambrisentan treatment on exercise-induced pulmonary hypertension in systemic sclerosis: a prospective single-center, open-label pilot study.

OBJECTIVE: Exercise-induced pulmonary hypertension (ePH) may represent an early, clinically relevant phase in the spectrum of pulmonary vascular disease. The purpose of this pilot study was to describe the changes in hemodynamics and exercise capacity in patients with systemic sclerosis (SSc) spectrum-associated ePH treated with open-label daily ambrisentan. METHODS: Patients were treated with ambrisentan, 5 mg or 10 mg once daily, for 24 weeks. At baseline and 24 weeks, patients with SSc spectrum disorders exercised in a supine position, on a lower extremity cycle ergometer. All patients had normal hemodynamics at rest. We defined baseline ePH as a mean pulmonary artery pressure of >30 mm Hg with maximum exercise and a transpulmonary gradient (TPG) of >15 mm Hg. The primary end point was change in pulmonary vascular resistance (PVR) with exercise. Secondary end points included an improvement from baseline in 6-minute walking distance, health-related quality of life assessments, and cardiopulmonary hemodynamics. RESULTS: Of the 12 enrolled patients, 11 completed the study. At 24 weeks there were improvements in mean exercise PVR (85.8 dynes × second/cm(5) ; P = 0.003) and mean distance covered during 6-minute walk (44.5 meters; P = 0.0007). Improvements were also observed in mean exercise cardiac output (1.4 liters/minute; P = 0.006), mean pulmonary artery pressure (-4.1 mm Hg; P = 0.02), and total pulmonary resistance (-93.0 dynes × seconds/cm(5) ; P = 0.0008). Three patients developed resting pulmonary arterial hypertension during the 24 weeks. CONCLUSION: Exercise hemodynamics and exercise capacity in patients with SSc spectrum-associated ePH improved over 24 weeks with exposure to ambrisentan. Placebo-controlled studies are needed to confirm whether this is a drug-related effect and to determine optimal therapeutic regimens for patients with ePH.

A computer-aided diagnosis system for quantitative scoring of extent of lung fibrosis in scleroderma patients.

OBJECTIVES: To evaluate an improved quantitative lung fibrosis score based on a computer-aided diagnosis (CAD) system that classifies CT pixels with the visual semi-quantitative pulmonary fibrosis score in patients with scleroderma-related interstitial lung disease (SSc-ILD). METHODS: High-resolution, thin-section CT images were obtained and analysed on 129 subjects with SSc-ILD (36 men, 93 women; mean age 48.8±12.1 years) who underwent baseline CT in the prone position at full inspiration. The CAD system segmented each lung of each patient into 3 zones. A quantitative lung fibrosis (QLF) score was established via 5 steps: 1) images were denoised; 2) images were grid sampled; 3) the characteristics of grid intensities were converted into texture features; 4) texture features classified pixels as fibrotic or non-fibrotic, with fibrosis defined by a reticular pattern with architectural distortion; and 5) fibrotic pixels were reported as percentages. Quantitative scores were obtained from 709 zones with complete data and then compared with ordinal scores from two independent expert radiologists. ROC curve analyses were used to measure performance. RESULTS: When the two radiologists agreed that fibrosis affected more than 1% or 25% of a zone or zones, the areas under the ROC curves for QLF score were 0.86 and 0.96, respectively. CONCLUSIONS: Our technique exhibited good accuracy for detecting fibrosis at a threshold of both 1% (i.e. presence or absence of pulmonary fibrosis) and a clinically meaningful threshold of 25% extent of fibrosis in patients with SSc-ILD.

Further delineation of Raine syndrome.

We report o a baby from an Arab family with Raine syndrome. The baby presented at birth with severe craniofacial anomalies including a wide anterior fontanelle, exophthalmos, severe depression of the nasal bridge with a hypoplastic midface, bilateral choanal atresia and a large protruding tongue. All the limbs were short and the thorax was small. Radiologically there was increased bone density in some bones, periosteal new bone formation and marked bowing of the femurs, tibiae, and ulnae. We suggest that osteosclerosis in Raine syndrome is not necessarily severe and generalized, and bowing of the long bones is another variable radiological feature of the syndrome.