• antineutrophil cytoplasmic antibody (anca) associated vasculitis (aav)
• churg-strauss syndrome
• dermatopathology
• eosinophilic granulomatosis with polyangiitis (egpa)
• granulomatosis with polyangiitis (gpa)
• panca/mpo (myeloperoxidase)-positive microscopic polyangiitis (mpa)
• polymyositis
• sle pathogenesis
• systemic lupus erythematosus
• vasculitis

• asthma
• churg-strauss syndrome
• eosinophilic granulomatosis with polyangiitis
• prevalence
• vasculitis

• Adolescent
• Adult
• Asthma/diagnosis
• Asthma/drug therapy
• Asthma/epidemiology
• Churg-Strauss Syndrome/diagnosis
• Churg-Strauss Syndrome/drug therapy
• Churg-Strauss Syndrome/epidemiology
• Granulomatosis with Polyangiitis/diagnosis
• Granulomatosis with Polyangiitis/drug therapy
• Granulomatosis with Polyangiitis/epidemiology
• Humans
• Patient Acceptance of Health Care
• Prevalence

Cardiac manifestations are common and life-threatening in eosinophilic granulomatosis with polyangiitis (EGPA), which remains poorly studied in China. We aim to investigate its clinical features, associated factors, treatment, and outcomes.

We reviewed the clinical records of 110 EGPA patients and examined the independent factors associated with cardiac manifestations using multivariate logistic regression. Receiver operating characteristic curves determined the cut-off values, and survival was calculated via Kaplan–Meier curves.

Cardiac involvement was present in 36.4% (40/110) of EGPA patients, which mainly manifested as pericardial effusion (16.4%, 18/110), myocardial involvement (13.6%, 15/110), and heart failure (8.2%, 9/110). The mean age was 42.1 ± 14.23 years with no female/male predominance. Compared with the cardiac-unaffected group, the cardiac-affected group showed a lower rate of biopsy-proved vasculitis (0% versus 20%, p = 0.002). The eosinophil count [odds ratio (OR) = 1.142, 95% confidence interval (CI) 1.029–1.267] was independently associated with cardiac manifestations in EGPA, with a cut-off value of 3.66 × 10⁹/L [area under the curve (AUC) = 0.692, p = 0.001]. Regarding treatment, the cardiac-affected group displayed a higher ratio of glucocorticoid pulse combined with intravenous cyclophosphamide (CYC-IV) (40% versus 21.4%, p = 0.037), and intravenous immunoglobulin combined with glucocorticoid plus CYC-IV (17.5% versus 4.3%, p = 0.035) than the control group. Outcomes (p = 0.131) and survival (p = 0.1972) were not significantly different between the groups.

In this single-center Chinese EGPA cohort, cardiac manifestations are observed in 36.4% of patients, which primarily presents as myocardial involvement, pericardial effusion, and heart failure, independently associated with eosinophil count. Glucocorticoid combined with cyclophosphamide is the treatment cornerstone for EGPA patients with cardiac manifestations.

• associated factors
• cardiac involvement
• cardiac manifestations
• eosinophilic granulomatosis with polyangiitis
• outcomes
• treatment

Secondary pulmonary hemorrhage increases the risk of mortality in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV); plasma exchange therapy may improve outcomes in these patients. We conducted a retrospective cohort study to investigate the effect of plasma exchange therapy on short-term prognoses in patients with pulmonary hemorrhage secondary to AAV.

This study utilized the Diagnosis Procedure Combination database, which is a nationwide inpatient database in Japan. We checked the abstract data and medical actions and identified the patients with pulmonary hemorrhage secondary to AAV who required proactive treatment between 2009 and 2014. To compare the in-hospital mortality, we performed propensity score matching between the plasma exchange and non-plasma exchange groups at a ratio of 1:1.

Of the 52,932 patients with AAV, 940 developed pulmonary hemorrhage as a complication. A total of 249 patients from 194 hospitals were eligible for the study. Propensity score matching at a ratio of 1:1 was performed, and 59 pairs were formed (plasma exchange group, n = 59; non-plasma exchange group, n = 59). A statistically significant difference was found in the all-cause in-hospital mortality between the plasma exchange and non-plasma exchange groups (35.6% vs. 54.2%; p = 0041; risk difference, −18.6; 95% confidence interval (CI), −35.4% to −0.67%).

Thus, plasma exchange therapy was associated with improved in-hospital mortality in patients with pulmonary hemorrhage secondary to AAV.

• Aged
• Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications
• Female
• Hemorrhage/mortality
• Hemorrhage/therapy
• Hospital Mortality
• Humans
• Lung Diseases/mortality
• Lung Diseases/therapy
• Male
• Plasma Exchange/methods
• Propensity Score
• Retrospective Studies
• Treatment Outcome

• a Grant-in-Aid for Research on Policy Planning and Evaluation from the Ministry of Health, Labour, and Welfare, Japan

Reduced level of arousal is commonly observed in medical admissions and may predict in-hospital mortality. Delirium and reduced level of arousal are closely related. We systematically reviewed and conducted a meta-analysis of studies in adult acute medical patients of the relationship between reduced level of arousal on admission and in-hospital mortality.

We conducted a systematic review (PROSPERO: CRD42016022048), searching MEDLINE and EMBASE. We included studies of adult patients admitted with acute medical illness with level of arousal assessed on admission and mortality rates reported. We performed meta-analysis using a random effects model.

From 23,941 studies we included 21 with 14 included in the meta-analysis. Mean age range was 33.4 - 83.8 years. Studies considered unselected general medical admissions (8 studies, n=13,039) or specific medical conditions (13 studies, n=38,882). Methods of evaluating level of arousal varied. The prevalence of reduced level of arousal was 3.1%-76.9% (median 13.5%). Mortality rates were 1.7%-58% (median 15.9%). Reduced level of arousal was associated with higher in-hospital mortality (pooled OR 5.71; 95% CI 4.21-7.74; low quality evidence: high risk of bias, clinical heterogeneity and possible publication bias).

Reduced level of arousal on hospital admission may be a strong predictor of in-hospital mortality. Most evidence was of low quality. Reduced level of arousal is highly specific to delirium, better formal detection of hypoactive delirium and implementation of care pathways may improve outcomes. Future studies to assess the impact of interventions on in-hospital mortality should use validated assessments of both level of arousal and delirium.

The online version of this article (10.1186/s12877-017-0661-7) contains supplementary material, which is available to authorized users.

• Altered mental status
• Delirium
• Glasgow Coma Scale
• Mortality
• Systematic review

• ANCA-associated
• Disease prevalence
• Eosinophilic granulomatosis
• Epidemiology
• Morbidity
• Polyangiitis
• Vasculitis