Inter-rater reliability and prognostic value of baseline Radiographic Assessment of Lung Edema (RALE) scores in observational cohort studies of inpatients with COVID-19.

OBJECTIVES: To reliably quantify the radiographic severity of COVID-19 pneumonia with the Radiographic Assessment of Lung Edema (RALE) score on clinical chest X-rays among inpatients and examine the prognostic value of baseline RALE scores on COVID-19 clinical outcomes. SETTING: Hospitalised patients with COVID-19 in dedicated wards and intensive care units from two different hospital systems. PARTICIPANTS: 425 patients with COVID-19 in a discovery data set and 415 patients in a validation data set. PRIMARY AND SECONDARY OUTCOMES: We measured inter-rater reliability for RALE score annotations by different reviewers and examined for associations of consensus RALE scores with the level of respiratory support, demographics, physiologic variables, applied therapies, plasma host-response biomarkers, SARS-CoV-2 RNA load and clinical outcomes. RESULTS: Inter-rater agreement for RALE scores improved from fair to excellent following reviewer training and feedback (intraclass correlation coefficient of 0.85 vs 0.93, respectively). In the discovery cohort, the required level of respiratory support at the time of CXR acquisition (supplemental oxygen or non-invasive ventilation (n=178); invasive-mechanical ventilation (n=234), extracorporeal membrane oxygenation (n=13)) was significantly associated with RALE scores (median (IQR): 20.0 (14.1-26.7), 26.0 (20.5-34.0) and 44.5 (34.5-48.0), respectively, p<0.0001). Among invasively ventilated patients, RALE scores were significantly associated with worse respiratory mechanics (plateau and driving pressure) and gas exchange metrics (PaO2/FiO2 and ventilatory ratio), as well as higher plasma levels of IL-6, soluble receptor of advanced glycation end-products and soluble tumour necrosis factor receptor 1 (p<0.05). RALE scores were independently associated with 90-day survival in a multivariate Cox proportional hazards model (adjusted HR 1.04 (1.02-1.07), p=0.002). We replicated the significant associations of RALE scores with baseline disease severity and mortality in the independent validation data set. CONCLUSIONS: With a reproducible method to measure radiographic severity in COVID-19, we found significant associations with clinical and physiologic severity, host inflammation and clinical outcomes. The incorporation of radiographic severity assessments in clinical decision-making may provide important guidance for prognostication and treatment allocation in COVID-19.

Radiographic Assessment of Lung Edema (RALE) Scores are Highly Reproducible and Prognostic of Clinical Outcomes for Inpatients with COVID-19.

INTRODUCTION: Chest imaging is necessary for diagnosis of COVID-19 pneumonia, but current risk stratification tools do not consider radiographic severity. We quantified radiographic heterogeneity among inpatients with COVID-19 with the Radiographic Assessment of Lung Edema (RALE) score on Chest X-rays (CXRs). METHODS: We performed independent RALE scoring by ≥2 reviewers on baseline CXRs from 425 inpatients with COVID-19 (discovery dataset), we recorded clinical variables and outcomes, and measured plasma host-response biomarkers and SARS-CoV-2 RNA load from subjects with available biospecimens. RESULTS: We found excellent inter-rater agreement for RALE scores (intraclass correlation co-efficient=0.93). The required level of respiratory support at the time of baseline CXRs (supplemental oxygen or non-invasive ventilation [n=178]; invasive-mechanical ventilation [n=234], extracorporeal membrane oxygenation [n=13]) was significantly associated with RALE scores (median [interquartile range]: 20.0[14.1-26.7], 26.0[20.5-34.0] and 44.5[34.5-48.0], respectively, p<0.0001). Among invasively-ventilated patients, RALE scores were significantly associated with worse respiratory mechanics (plateau and driving pressure) and gas exchange metrics (PaO2/FiO2 and ventilatory ratio), as well as higher plasma levels of IL-6, sRAGE and TNFR1 levels (p<0.05). RALE scores were independently associated with 90-day survival in a multivariate Cox proportional hazards model (adjusted hazard ratio 1.04[1.02-1.07], p=0.002). We validated significant associations of RALE scores with baseline severity and mortality in an independent dataset of 415 COVID-19 inpatients. CONCLUSION: Reproducible assessment of radiographic severity revealed significant associations with clinical and physiologic severity, host-response biomarkers and clinical outcome in COVID-19 pneumonia. Incorporation of radiographic severity assessments may provide prognostic and treatment allocation guidance in patients hospitalized with COVID-19.

Chest Radiograph Severity and Its Association With Outcomes in Subjects With COVID-19 Presenting to the Emergency Department.

BACKGROUND: Severity of radiographic abnormalities on chest radiograph in subjects with COVID-19 has been shown to be associated with worse outcomes, but studies are limited by different scoring systems, sample size, subject age, and study duration. Data regarding the longitudinal evolution of radiographic abnormalities and its association with outcomes are scarce. We sought to evaluate these questions using a well-validated scoring system (the Radiographic Assessment of Lung Edema [RALE] score) using data over 6 months from a large, multihospital health care system. METHODS: We collected clinical and demographic data and quantified radiographic edema on chest radiograph obtained in the emergency department (ED) as well as on days 1-2 and 3-5 (in those admitted) in subjects with a nasopharyngeal swab positive for SARS-CoV-2 by polymerase chain reaction (PCR) visiting the ED for coronavirus disease 2019 (COVID)-19-related complaints between March-September 2020. We examined the association of baseline and longitudinal evolution of radiographic edema with severity of hypoxemia and clinical outcomes. RESULTS: Eight hundred and seventy subjects were included (median age 53.6; 50.8% female). Inter-rate agreement for RALE scores was excellent (interclass correlation coefficient 0.84 [95% CI 0.82-0.87], P < .001). RALE scores correlated with hypoxemia as quantified by SpO2 /FIO2 (r = -0.42, P < .001). Admitted subjects had higher RALE scores than those discharged (6 [2-11] vs 0 [0-3], P < .001). An increase of RALE score ≥ 4 was associated with worse 30-d survival (P = .006). Larger increases in the RALE score were associated with worse survival. CONCLUSIONS: The RALE score was reproducible and easily implementable in adult subjects presenting to the ED with COVID-19. Its association with physiologic parameters and outcomes at baseline and longitudinally makes it a readily available tool for prognostication and early ICU triage, particularly in patients with worsening radiographic edema.

Case of Missing Plastic: Foreign Body Bronchiectasis.

Bronchiectasis is a well-known entity where the airways abnormally dilate losing their natural function. Most common causes of non-cytic fibrosis bronchiectasis in the middle age group include secondary immunodeficiency, aspiration, and allergic bronchopulmonary aspergillosis (ABPA). Obstructive foreign body is an uncommon cause of bronchiectasis and is often a missed diagnosis in a localized disease. Foreign bodies can be missed making the diagnosis and treatment more challenging and hence foreign body bronchiectasis should be considered in patients presenting with focal disease. Here we describe a patient with a retained foreign body that was discovered post lobectomy during gross pathological examination of the specimen with no significant aspiration history, non-diagnostic imaging of the chest and negative bronchoscopy.

Pulmonary Artery Dimensions as a Prognosticator of Transplant-Free Survival in Scleroderma Interstitial Lung Disease.

BACKGROUND: Systemic sclerosis is a chronic debilitating autoimmune disease characterized by endothelial dysfunction and multi-organ fibrosis. Interstitial lung disease, a common manifestation of SSc, is termed scleroderma-related interstitial lung disease (SSc-ILD) and along with pulmonary hypertension contributes to a majority of deaths in SSc. SSc-ILD patients frequently develop pulmonary hypertension, which prognosticates a poorer outcome. We investigated pulmonary artery dimensions as an outcome predictor in patients with SSc-ILD. METHODS: A retrospective chart review abstracting data from SSc-ILD patients evaluated at a large tertiary care center was performed. HRCT imaging was reviewed and pulmonary artery (PA) and ascending aorta (Ao) diameters were measured for calculation of the PA:Ao ratio. Additionally, demographics, vital signs, spirometric parameters, comorbidities, and mean pulmonary artery pressures were collected when available. Outcome analysis with lung transplant or death events within 4 years based on pulmonary artery size as well as PA:Ao ratio was performed. RESULTS: 70 SSc-ILD patients were identified. Mean pulmonary artery diameter and PA:Ao ratio was 31.17 and 1.07 mm, respectively. Patients with a pulmonary artery diameter ≥32 mm had higher risk of lung transplantation or death (p < 0.001) within 4 years. Patients with a PA:Ao ratio ≥1.1 also had higher risk of lung transplantation or death (p < 0.001) within 4 years. Unadjusted outcomes analyses also identified PA:Ao ratio ≥1.1 as an independent outcome predictor (hazard ratio 3.30, p < 0.001). CONCLUSIONS/CLINICAL IMPLICATIONS: In SSc-ILD patients, a PA:Ao ratio ≥1.1 is associated with higher risk of lung transplant or death. These data suggest that PA:Ao dimension may be used for prognostication in SSc-ILD.

Combined Intrathoracic and Subcutaneous Splenosis Discovered 51 Years after Abdominal Trauma.

Splenosis is a rare condition that results from the autotransplantation of splenic parenchyma into unexpected locations such as the abdomen or subcutaneous tissue. In the presence of coexisting injury to the diaphragm intrathoracic transplantation can occur emerging as single or multiple pleural-based masses. This occurs after traumatic rupture of the spleen and is usually asymptomatic, only to be discovered incidentally on routine thoracic or abdominal imaging. To our knowledge this is the third documented case of combined intrathoracic and subcutaneous splenosis found in English literature. This occurred in a 71-year-old male involved in a motor vehicle accident at age 19 requiring urgent splenectomy. He has a significant cigarette smoking history and was referred to our hospital for further evaluation of an abnormality seen on shoulder X-ray.

Food Particle Aspiration Associated with Hemorrhagic Shock: A Diagnostic Dilemma.

The hemodynamic compromise caused by a large aspirated food particle in the airway can become the focus of medical attention and a distraction from rare but fatal Heimlich maneuver related injuries after an incident of food aspiration. We herein present a case of an 84-year-old man who was brought to the emergency department after an episode of choking at a restaurant followed by several failed Heimlich maneuver attempts. Despite relieving the airway obstruction by extracting a large piece of steak from the airway, the patient remained hypotensive and required continued hemodynamic support. Repeated laboratory tests within 24 hrs of aspiration showed a significant decline in the hemoglobin level. A computed tomography (CT) scan of the abdomen and pelvis showed a lacerated liver with a large subcapsular hematoma draining into the pelvis. Conclusion. Hepatic rupture is a rare complication of Heimlich maneuver; this paper represents the second case report in the literature. It emphasizes the necessity of early identification and surveillance of fatal Heimlich maneuver complications in a high risk population.

Vitamin D deficiency is associated with pulmonary dysfunction in cystic fibrosis.

BACKGROUND: Vitamin D deficiency is common in CF. Whether vitamin D affects pulmonary function in CF is unknown. METHODS: Data were abstracted from clinically stable CF patients who had pulmonary function studies and serum 25-hydroxyvitamin D [25(OH)D, ng/ml] levels drawn within 2 months of each other. Findings were adjusted for multiple variables known to affect pulmonary function in CF. RESULTS: Enrollees totaled 597. Overall mean 25(OH)D level was 29.6±12.8 ng/ml (SD). Serum 25(OH)D levels showed a significant correlation with forced expiratory volume in 1s (FEV1) % predicted (r=0.20, p<0.0001) and forced vital capacity % predicted (r=0.13, p=0.0019). Multivariate analysis revealed that serum 25(OH)D remained an independent predictor of FEV1 % predicted even after controlling for multiple other factors known to affect CF lung function. CONCLUSIONS: Serum 25(OH)D levels are significantly associated with pulmonary function in CF. Further study is required to determine whether this association is causal.

Pulmonary Sequestration: A 29 Patient Case Series and Review.

INTRODUCTION: Pulmonary sequestration also known as bronchopulmonary sequestration is a rare disease, with very few case series reviewed in literature. In this study, we review the demographics, presentation, imaging and treatment of pulmonary sequestration in 29 patients from our institution, and provide comparison data from previously published series with an overview of the disease history. MATERIALS AND METHODS: Records reviewed for all patients evaluated and treated in our institution with a pathological proven diagnosis of pulmonary sequestration from January 2004 through December 2013. Collected data included demographics, clinical presentation, diagnostic imaging, location of the lesion, type of sequestration, and subsequent treatment. RESULTS: Of the 29 patients reviewed 8 (28%) were children 0-2 years, 1 adolescent age 17, and 20 (69%) adults 21-70 years with a mean age of 42 among adults. Systemic arterial supply to the sequestered segment was demonstrated with computed tomographic angiography (CTA) in 25 patients (86%). In 19 patients (66%), the sequestered segment was located in the left lower lobe, and 16 (55%) were intralobar. CONCLUSION: Diagnostic delays of pulmonary sequestration were common among the adult population as the presenting symptoms often mimicked other common pulmonary diseases, such as pneumonia and asthma. These findings were consistent among previously published series. CTA was the preferred imaging modality for preoperative planning with high sensitivity and specificity in identifying the lesion.

Vanishing Lung Syndrome in a Patient with HIV Infection and Heavy Marijuana Use.

Vanishing lung syndrome (VLS) is a rare and distinct clinical syndrome that usually affects young men. VLS leads to severe progressive dyspnea and is characterized by extensive, asymmetric, peripheral, and predominantly upper lobe giant lung bullae. Case reports have suggested an additive role of marijuana use in the development of this disease in young male tobacco smokers. We herein report a case of a 65-year-old Hispanic male previously diagnosed with severe emphysema and acquired immune deficiency syndrome (AIDS), with a history of intravenous heroin use and active marijuana smoking who presents to the emergency department with severe progressive shortness of breath he was found to have multiple large subpleural bullae occupying more than one-third of the hemithorax on chest computerized tomography (CT), characteristic of vanishing lung syndrome. The patient was mechanically ventilated and later developed a pneumothorax requiring chest tube placement and referral for surgical bullectomy. Surgical bullectomy has shown high success rates in alleviating the debilitating symptoms and preventing the life threatening complications of this rare syndrome. This case further emphasizes the importance of recognizing VLS in patients with severe emphysema and heavy marijuana smoking.

Cystic fibrosis and pregnancy in the modern era: a case control study.

BACKGROUND: Increasingly, women with cystic fibrosis become pregnant. Outcomes of these women need further study particularly in the setting of improved survival in CF. METHODS: We performed a case-control study of pregnant CF women including 22 matched pairs with an average follow-up of 4.5 years. Nutritional outcomes, changes in lung function, and exacerbation rates were compared. RESULTS: Matched pairs were similar in age, sweat chloride, FEV1 and FVC % predicted, BMI, and diabetes status. Change in BMI, FEV1 and FVC % predicted at the end of pregnancy and at last follow-up were similar between groups. Moreover, rates of exacerbation before, during and after pregnancy were similar. On multivariable analysis pregnancy had no effect on change in lung function over the study period. Significant predictors of decline included higher pre-pregnancy lung function and pancreatic insufficiency. CONCLUSIONS: Pregnancy does not lead to immediate or medium-term adverse effects for CF patients.

Unusual presentation and location pleural malignant mesothelioma.

Pleural malignant mesothelioma is an uncommon tumour of the thorax. We report two cases: a patient with atypically isolated location of the tumour at the right hilum and a young female patient. The histopathological and radiological features are reviewed with reference to relevant literature.