Management of cardiac sarcoidosis in the United States: a Delphi study.
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ABSTRACT:
SUBMITTER: Hamzeh NY
PROVIDER: S-EPMC3416033 | biostudies-other | 2012 Jan
REPOSITORIES: biostudies-other
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Project description:The clinical presentation and outcome of sarcoidosis varies by race. However, the race difference in mortality outcome remains largely unknown.We studied mortality related to sarcoidosis from 1999 through 2010 by examining data on multiple causes of death from the National Center for Health Statistics. We compared the comorbid conditions between sarcoidosis-related deaths with deaths caused by car accidents (previously healthy control subjects) and rheumatoid arthritis (chronic disease control subjects) in both African Americans and Caucasians.From 1999 through 2010, sarcoidosis was reported as an immediate cause of death in 10,348 people in the United States with a combined overall mean age-adjusted mortality rate of 2.8 per 1 million person-years. Of these, 6,285 were African American and 3,984 Caucasian. The age-adjusted mortality rate for African Americans was 12 times higher than for Caucasians. African Americans died at an earlier age than Caucasians. African Americans living in the District of Columbia and North Carolina and Caucasians living in Vermont had higher mortality rates. Although the total sarcoidosis age-adjusted mortality rate had not changed over the 12 year period studied, this rate increased for Caucasians (R = 0.747, P = .005) but not for African Americans. Compared with the control groups, pulmonary hypertension was significantly more common in individuals with sarcoidosis.This nationwide population-based study exposes a significant difference in ethnicity and sex among people dying of sarcoidosis in the United States. Pulmonary hypertension investigation should be considered in all patients with sarcoidosis, especially African Americans.
| S-EPMC4314818 | biostudies-other
Project description:BackgroundSarcoidosis is a systemic granulomatous disorder of unknown cause that occurs among men and women of all races. In the United States, black women are most frequently and most severely affected. There have been few epidemiologic studies of sarcoidosis focusing on black women.MethodsIn this article, we present data on incidence, prevalence, and clinical characteristics of sarcoidosis among participants in the Black Women's Health Study, a cohort study of 59,000 black women from across the United States. Data on incident disease and potential risk factors are obtained through biennial questionnaires. Follow-up has been > 80% through six completed cycles.ResultsThere were 685 prevalent cases of sarcoidosis at baseline in 1995 and 435 incident cases reported during 611,585 person-years of follow-up through 2007, for an average annual incidence rate of 71/100,000 and a current prevalence of 2.0%. The sarcoid diagnosis was confirmed in 96% of self-reported cases for whom medical records or physician checklists were obtained. The most frequently affected site was the lung. Most patients also had extrapulmonary involvement, with the most common sites being lymph nodes, skin, and eyes. Prednisone had the highest prevalence of use, followed by inhaled corticosteroids.ConclusionsThis study confirms previous reports of high incidence and prevalence of sarcoidosis among black women, as well as the extent of extrapulmonary disease, frequent need for steroid therapy, and comorbid conditions in this population. The prospective identification of sarcoidosis cases from a defined population will enable a valid assessment of risk factors for incident disease as follow-up continues.
| S-EPMC3014690 | biostudies-literature
Project description:RationaleIt has been nearly 20 years since sarcoidosis mortality was examined at the population level in the United States.ObjectivesTo examine mortality rates and underlying causes of death among United States decedents with sarcoidosis from 1988-2007.MethodsWe used data from the National Center for Health Statistics to (1) calculate age-adjusted sarcoidosis-associated mortality rates; (2) examine how those rates differ by age, sex, and race and ethnicity; and (3) determine underlying causes of death among sarcoidosis decedents.Measurements and main resultsFrom 1988-2007, there were 46,450,489 deaths in the United States and 23,679 decedents with sarcoidosis mentioned on their death certificates. Over this time, the age-adjusted, sarcoidosis-related mortality rate increased 50.5% in women and 30.1% in men. The greatest absolute increase in death rates was among non-Hispanic black females. Regardless of sex or race, mortality rates climbed most in decedents 55 years or older. The most common cause of death was sarcoidosis itself. Younger sarcoidosis decedents with pulmonary fibrosis were more likely to be black than white, and younger sarcoidosis decedents were more likely than similarly aged decedents in the general population to have a cardiac cause contribute to death.ConclusionsFrom 1988-2007, sarcoidosis-related mortality rates increased significantly, particularly in non-Hispanic black females aged 55 years or older. The underlying cause of death in most patients with sarcoidosis was the disease itself. Among young sarcoidosis decedents, those with pulmonary fibrosis or a cardiac cause contributing to death were more likely to be black than white.
| S-EPMC3137141 | biostudies-literature
Project description:BackgroundPatients with cardiac sarcoidosis are at increased risk of ventricular tachycardia/fibrillation.ObjectiveWe tested the hypothesis that the wearable cardioverter defibrillator can be used to mitigate the risk of sudden cardiac death among cardiac sarcoidosis patients.MethodsA retrospective review of the commercial database identified cardiac sarcoidosis patients who wore the wearable cardioverter defibrillator. Evidence for cardiac sarcoidosis diagnosis as well as demographic, co-morbidity and left ventricular ejection fraction were provided by patient clinical records. Clinical data also included daily wearable cardioverter defibrillator wear, shock treatment and survival information.ResultsThe wearable cardioverter defibrillator was worn by 46 cardiac sarcoidosis patients, 24 (52%) male. The median age was 48 years and median left ventricular ejection fraction was 30%. The wearable cardioverter defibrillator was worn a median of 23.6 hours each day. There were 11 ventricular tachycardia/fibrillation episodes occurring in 10 (22%) patients. Ventricular tachycardia/fibrillation occurred over a range of 1 to 79 days, median 24 days. First-shock success for conversion of ventricular tachycardia/fibrillation was 100%. Patient survival 24 hours after shock treatment was 100%. Follow up to determine the reason for discontinuing wearable cardioverter defibrillator use indicated that among shocked patients 7 received an implantable cardioverter defibrillator, 1 patient was admitted to the hospital ending in death 2 weeks after discontinuing wearable cardioverter defibrillator use, and 2 patients were lost to follow up. Among the not shocked patients, there were 16 who received an implantable cardioverter defibrillator while 7 achieved improved left ventricular ejection fraction.ConclusionManagement of sudden cardiac death among cardiac sarcoidosis patients was aided by the wearable cardioverter defibrillator resulting in successful termination of ventricular tachycardia/fibrillation upon delivery of shock.
| S-EPMC5864098 | biostudies-literature
Project description:PURPOSE OF REVIEW:In this state-of-the-art review, we highlight our current understanding of diagnosis, assessment, and management of cardiac sarcoidosis (CS), focusing on recently published data and expert consensus statement guidelines. RECENT FINDINGS:Academic interest in cardiac sarcoidosis research has increased over the past decade along with increased clinical awareness among clinicians. In 2014, the Heart Rhythm Society published the first expert consensus statement on diagnosing and managing arrhythmias associated with CS. Cardiac magnetic resonance has emerged as a valuable tool both for diagnosing CS and predicting risk of life-threatening ventricular arrhythmias based on burden of late gadolinium enhancement. Cardiac fluorodeoxyglucose-positron emission tomography now plays a role in diagnosis, risk stratification, and assessing response to immunosuppressive therapy. Collaborative, multidisciplinary research efforts are needed to further our understanding of this rare, complex disease. Two large multicenter prospective registries-the international Cardiac Sarcoidosis Consortium and the Canadian Cardiac Sarcoidosis Research Group-are enrolling patients to help provide insights into the natural history of the disease and current treatment strategies. Future research should focus on randomized controlled trials comparing different treatment strategies and identifying and testing novel therapeutic agents.
| S-EPMC7092931 | biostudies-literature
Project description:BackgroundPrognostic factors are lacking in cardiac sarcoidosis (CS), and the effects of immunosuppressive treatments are unclear.ObjectivesTo identify prognostic factors and to assess the effects of immunosuppressive drugs on relapse risk in patients presenting with CS.MethodsFrom a cohort of 157 patients with CS with a median follow-up of 7 years, we analysed all cardiac and extra-cardiac data and treatments, and assessed relapse-free and overall survival.ResultsThe 10-year survival rate was 90% (95% CI, 84-96). Baseline factors associated with mortality were the presence of high degree atrioventricular block (HR, 5.56, 95% CI 1.7-18.2, p = 0.005), left ventricular ejection fraction below 40% (HR, 4.88, 95% CI 1.26-18.9, p = 0.022), hypertension (HR, 4.79, 95% CI 1.06-21.7, p = 0.042), abnormal pulmonary function test (HR, 3.27, 95% CI 1.07-10.0, p = 0.038), areas of late gadolinium enhancement on cardiac magnetic resonance (HR, 2.26, 95% CI 0.25-20.4, p = 0.003), and older age (HR per 10 years 1.69, 95% CI 1.13-2.52, p = 0.01). The 10-year relapse-free survival rate for cardiac relapses was 53% (95% CI, 44-63). Baseline factors that were independently associated with cardiac relapse were kidney involvement (HR, 3.35, 95% CI 1.39-8.07, p = 0.007), wall motion abnormalities (HR, 2.30, 95% CI 1.22-4.32, p = 0.010), and left heart failure (HR 2.23, 95% CI 1.12-4.45, p = 0.023). After adjustment for cardiac involvement severity, treatment with intravenous cyclophosphamide was associated with a lower risk of cardiac relapse (HR 0.16, 95% CI 0.033-0.78, p = 0.024).ConclusionsOur study identifies putative factors affecting morbidity and mortality in cardiac sarcoidosis patients. Intravenous cyclophosphamide is associated with lower relapse rates.
| S-EPMC7500618 | biostudies-literature
Project description:BackgroundPresentation of life-threatening arrhythmias concomitantly with a new-onset non-ischaemic cardiomyopathy raises concern for an inflammatory cardiomyopathy such as cardiac sarcoidosis or cardiac manifestations of connective tissue disease. Comprehensive workup for specific aetiologies may be unrevealing except for signs of myocardial inflammation identified on cardiac positron emission tomography (PET). Here, we present five cases of such subjects and their clinical course.Case summaryWe collected clinical, imaging, pathological, and follow-up data of five subjects presenting with arrhythmias and unexplained new-onset cardiomyopathy. Mean age was 56.2 ± 5.8 years. Three subjects presented with ventricular tachycardia and two with atrial arrhythmias. Echocardiography showed a mean left ventricular ejection fraction of 37 ± 9%. Significant coronary artery disease was ruled out in all cases as the cause of the cardiomyopathy. All patients underwent cardiac magnetic resonance imaging (MRI) and PET scan at presentation and follow-up. In all patients, cardiac MRI revealed hyperenhancement in epicardial and mid-myocardial pattern in a non-coronary distribution, while PET scan revealed fluorodeoxyglucose (FDG) mismatch defects in multiple foci in a non-coronary distribution. Right ventricular biopsy was obtained in all patients and revealed interstitial fibrosis and cardiomyocyte hypertrophy. On median follow-up of 210 days, all subjects had improvement in both heart failure symptoms and arrhythmias and repeat PET in four out of five patients showed decreased inflammation.DiscussionA high level of suspicion for inflammatory cardiomyopathy is needed in patients presenting with new unexplained cardiomyopathy and arrhythmias. A cardiac FDG-PET should be considered for diagnosis if cardiac inflammation is in the differential. This can inform further decisions regarding targeted immunomodulation therapy that may be helpful in this cohort.
| S-EPMC7501935 | biostudies-literature
Project description:Individual differences in peripheral blood transcriptomes in older adults as a function of demographic, socio-economic, psychological, and health history characteristics.
2015-11-15 | GSE68526 | GEO
Project description:BACKGROUND:Recent trends of hospitalizations and in-hospital mortality are not well defined in sarcoidosis. We examined aforementioned trends and prevalence of cardiovascular manifestations and explored rates of implantable cardioverter-defibrillator implantation in hospitalizations with sarcoidosis. METHODS AND RESULTS:Using data from the National Inpatient Sample, a retrospective population cohort from 2005 to 2014 was studied. To identify sarcoidosis, an International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) diagnosis code was used. We excluded hospitalizations with myocardial infarction, coronary artery disease, and ischemic cardiomyopathy. Cardiovascular manifestations were defined by the presence of diagnosis codes for conduction disorders, arrhythmias, heart failure, nonischemic cardiomyopathy, and pulmonary hypertension. A total of 609 051 sarcoidosis hospitalizations were identified, with an age of 55±14 years, 67% women, and 50% black. The number of sarcoidosis hospitalizations increased from 2005 through 2014 (138 versus 175 per 100 000, Ptrend<0.001). We observed declining trends of unadjusted in-hospital mortality (6.5 to 4.9 per 100 sarcoidosis hospitalizations, Ptrend<0.001). Overall ?31% (n=188 438) of sarcoidosis hospitalizations had coexistent cardiovascular manifestations of one or more type. Heart failure (?16%) and arrhythmias (?15%) were the most prevalent cardiovascular manifestations. Rates of implantable cardioverter-defibrillator placement were ?7.5 per 1000 sarcoidosis hospitalizations (Ptrend=0.95) during the study period. Black race was associated with 21% increased risk of in-hospital mortality (odds ratio, 1.21; 95% confidence interval, 1.16-1.27 [P<0.001]). CONCLUSIONS:Sarcoidosis hospitalizations have increased over the past decade with a myriad of coexistent cardiovascular manifestations. Black race is a significant predictor of in-hospital mortality, which is declining. Further efforts are needed to improve care in view of low implantable cardioverter-defibrillator rates in sarcoidosis.
| S-EPMC5850171 | biostudies-literature
Project description:ObjectiveThe aim of this study was to examine differences between general and pediatric emergency departments (PEDs) in adherence to the American Academy of Pediatrics bronchiolitis management guidelines.MethodsWe conducted a nationally representative study of ED visits by infants younger than 24 months with bronchiolitis from 2002 to 2011 using the National Hospital Ambulatory Medical Care Survey. Diagnostic testing (complete blood counts, radiographs) and medication use (albuterol, corticosteroids, antibiotics and intravenous fluids) in general emergency departments (GEDs) were compared with those in PEDs before and after 2006 American Academy of Pediatrics guideline publication. Weighted percentages were compared, and logistic regression evaluated the association between ED type and resource use.ResultsOf more than 2.5 million ED visits for bronchiolitis from 2002 to 2011, 77.3% occurred in GEDs. General emergency departments were more likely to use radiography (62.7% vs 42.1%; adjusted odds ratio [aOR], 2.4; 95% confidence interval [CI], 1.4-4.1), antibiotics (41.3% vs 18.8%; aOR, 2.8; 95% CI, 1.5-5.2), and corticosteroids (24.3% vs 12.5%; aOR, 2.1; 95% CI, 1.0-4.5) compared with PEDs. Compared with preguideline, after guideline publication PEDs had a greater decrease in radiography use (-19.7%; 95% CI, -39.3% to -0.03%) compared with GEDs (-12.2%; 95% CI, -22.3% to -2.1%), and PEDs showed a significant decline in corticosteroid use (-12.4%; 95% CI, -22.1% to -2.8%), whereas GEDs showed no significant decline (-4.6%; 95% CI, -13.5% to 4.3%).ConclusionsThe majority of ED visits for bronchiolitis in the United States occurred in GEDs, yet GEDs had increased use of radiography, corticosteroids, and antibiotics and did not show substantial declines with national guideline publication. Given that national guidelines discourage the use of such tests and treatments in the management of bronchiolitis, efforts are required to decrease ED use of these resources in infants with bronchiolitis, particularly in GEDs.
| S-EPMC5654708 | biostudies-literature