Objective. Primary Sjögren's syndrome (SS) may lead to lymphoproliferative disease (LPD) and death in certain patients. We sought to determine the incidence and predictors of adverse long-term outcomes to achieve a rational predictive classification of the syndrome.Methods. Predictive modeling was performed in a cohort of 723 consecutive patients with primary SS (587 newly diagnosed [incident] cases and 136 prevalent cases).Results. During 4,384 person-years of followup, we recorded 39 deaths (7 due to lymphoma) and 38 diagnoses of LPD. The standardized mortality ratio was 1.15 (95% confidence interval [95% CI] 0.86-1.73) compared with the general population of Greece. In incident cases, the probability of LPD was 2.6% at 5 years and 3.9% at 10 years. Mortality rates were significantly higher in patients with low C4 levels at the first study visit (hazard ratio [HR] 4.39, 95% CI 2.18-8.83). LPD was independently predicted by the presence of parotid enlargement (HR 5.21, 95% CI 1.76-15.4), palpable purpura (HR 4.16, 95% CI 1.65-10.5), and low C4 levels (HR 2.40, 95% CI 0.99-5.83) at the first study visit. All patients who eventually developed lymphoma resulting in death during the followup period had either low C4 levels or palpable purpura at the first study visit. Training-validation split-cohort modeling confirmed the predictive importance of low C4 levels and palpable purpura, both of which were present in 20.9% of patients at their first visit.Conclusions. In patients with primary SS, 1 in 5 deaths is attributable to lymphoma. The presence of palpable purpura and low C4 levels at the first visit adequately distinguishes high-risk patients (type I primary SS) from patients with an uncomplicated disease course (type II [low-risk] primary SS). Primary Sjögren's syndrome (SS), also known as autoimmune epitheliitis (1,2), is characterized by dry eyes and dry mouth. It affects ϳ0.2-1.0% of the general female population (3). The most worrying complication is the development of lymphoproliferative disease (LPD), most commonly lymphoma (4). Nevertheless, there are no specific data concerning the incidence of such serious sequelae and their impact on patient survival. Studies of primary SS have been limited by the sample size (5-7), and the largest studies have covered only a few hundred person-years of followup. Moreover, since the syndrome affects mostly middle-aged women, in whom the death rate is low, mortality studies require a large number of person-years to capture a meaningful number of deaths.Over the last 20 years, a total of 723 consecutive patients who have met strict criteria for a diagnosis of definite primary SS have been followed up at the University of Ioannina and the University of Athens. In the present study, we systematically evaluated the longterm outcomes of this cohort and performed predictive modeling for its major outcomes in order to identify high-risk patients. Meticulous efforts were made to ensure that information on the current status of all these patients would be retrieved. The resulti...

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In DCM, the presence of septal LGE is associated with a large increase in the risk of death and SCD events, even when the extent is small. SCD risk is greatest with concomitant septal and free-wall LGE. The incremental value of LGE extent beyond small amounts and LGE pattern is limited.

ImportancePost−COVID-19 condition (PCC) is a complex heterogeneous disorder that has affected the lives of millions of people globally. Identification of potential risk factors to better understand who is at risk of developing PCC is important because it would allow for early and appropriate clinical support.ObjectiveTo evaluate the demographic characteristics and comorbidities that have been found to be associated with an increased risk of developing PCC.Data sourcesMedline and Embase databases were systematically searched from inception to December 5, 2022.Study SelectionThe meta-analysis included all published studies that investigated the risk factors and/or predictors of PCC in adult (≥18 years) patients.Data Extraction and SynthesisOdds ratios (ORs) for each risk factor were pooled from the selected studies. For each potential risk factor, the random-effects model was used to compare the risk of developing PCC between individuals with and without the risk factor. Data analyses were performed from December 5, 2022, to February 10, 2023.Main Outcomes and MeasuresThe risk factors for PCC included patient age; sex; body mass index, calculated as weight in kilograms divided by height in meters squared; smoking status; comorbidities, including anxiety and/or depression, asthma, chronic kidney disease, chronic obstructive pulmonary disease, diabetes, immunosuppression, and ischemic heart disease; previous hospitalization or ICU (intensive care unit) admission with COVID-19; and previous vaccination against COVID-19.ResultsThe initial search yielded 5334 records of which 255 articles underwent full-text evaluation, which identified 41 articles and a total of 860 783 patients that were included. The findings of the meta-analysis showed that female sex (OR, 1.56; 95% CI, 1.41-1.73), age (OR, 1.21; 95% CI, 1.11-1.33), high BMI (OR, 1.15; 95% CI, 1.08-1.23), and smoking (OR, 1.10; 95% CI, 1.07-1.13) were associated with an increased risk of developing PCC. In addition, the presence of comorbidities and previous hospitalization or ICU admission were found to be associated with high risk of PCC (OR, 2.48; 95% CI, 1.97-3.13 and OR, 2.37; 95% CI, 2.18-2.56, respectively). Patients who had been vaccinated against COVID-19 with 2 doses had a significantly lower risk of developing PCC compared with patients who were not vaccinated (OR, 0.57; 95% CI, 0.43-0.76).Conclusions and RelevanceThis systematic review and meta-analysis demonstrated that certain demographic characteristics (eg, age and sex), comorbidities, and severe COVID-19 were associated with an increased risk of PCC, whereas vaccination had a protective role against developing PCC sequelae. These findings may enable a better understanding of who may develop PCC and provide additional evidence for the benefits of vaccination.Trial RegistrationPROSPERO Identifier: CRD42022381002

A polymorphism of the human angiotensin-1-converting enzyme (ACE) gene has been identified in which the presence (insertion, I allele) of a 287-bp fragment rather than the absence (deletion, D allele) is associated with lower ACE activity. Several recent studies have shown an association of the I allele with endurance performance, it being found with excess frequency in elite distance runners, rowers and mountaineers. Other workers using heterogeneous cohorts of athletes from mixed sporting disciplines have found no such association. An increasing linear trend of I allele frequency with the distance run amongst Olympic runners and an excess of the D allele amongst sprinters led us to examine whether the ratio of I and D alleles in swimmers competing over different distances would also vary. Swimmers (n=120) from the European and Commonwealth championships and an American college team had their ACE genotype determined and their gene and allele frequencies compared with several control groups, the most closely age-matched of which were 1,248 military recruits. Of the 103 Caucasians, there was a significant excess of the D allele compared with this control group only in the truly elite swimmers of the European and Commonwealth championships (P=0.004). This association remained in those competing over shorter distances (P=0.005 for 400 m and below) but not in the longer events. These findings were confirmed in three further large control groups. A population association study testing whether a genetic marker (the ACE I/D polymorphism) occurs more frequently in cases (elite athletes) than in controls therefore requires a homogeneous cohort of subjects from the same sporting discipline.

Aims The aim of this study is to evaluate temporal trends, treatment, and clinical outcomes of patients who present with an acute myocardial infarction (AMI) and have a current or historical diagnosis of cancer, according to cancer type and presence of metastases. Methods and results Data from 6 563 255 patients presenting with an AMI between 2004 and 2014 from the US National Inpatient Sample (NIS) database were analysed. A total of 5 966 955 had no cancer, 186 604 had current cancer, and 409 697 had a historical diagnosis of cancer. Prostate, breast, colon, and lung cancer were the four most common types of cancer. Patients with cancer were older with more comorbidities. Differences in invasive treatment were noted, 43.9% received percutaneous coronary intervention (PCI) in patients without cancer, whilst only 21.0% of patients with lung cancer received PCI. Lung cancer was associated with the highest in-hospital mortality [odds ratio (OR) 2.71, 95% confidence interval (CI) 2.62–2.80], major adverse cardiovascular and cerebrovascular complications (OR 2.38, 95% CI 2.31–2.45), and stroke (OR 1.91, 95% CI 1.80–2.02), while colon cancer was associated with highest risk of bleeding (OR 2.82, 95% CI 2.68–2.98). Irrespective of the type of cancer, presence of metastasis was associated with worse in-hospital outcomes, and historical cancer did not adversely impact on survival (OR 0.90, 95% CI 0.89–0.91). Conclusion A concomitant cancer diagnosis is associated with a conservative medical management strategy for AMI, and worse clinical outcomes, compared to patients without cancer. Survival and clinical outcomes in the context of AMI vary significantly according to the type of cancer and metastasis status. The management of this high-risk group is challenging and requires a multidisciplinary and patient-centred approach to improve their outcomes.

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Purpose of Review The role of renin-angiotensin-aldosterone system (RAAS) inhibitors, notably angiotensin-converting enzyme inhibitors (ACEi) or angiotensin receptor blockers (ARBs), in the COVID-19 pandemic has not been fully evaluated. With an increasing number of COVID-19 cases worldwide, it is imperative to better understand the impact of RAAS inhibitors in hypertensive COVID patients. PubMed, Embase and the pre-print database Medrxiv were searched, and studies with data on patients on ACEi/ARB with COVID-19 were included. Random effects models were used to estimate the pooled mean difference with 95% confidence interval using Open Meta[Analyst] software. Recent Findings A total of 28,872 patients were included in this meta-analysis. The use of any RAAS inhibition for any conditions showed a trend to lower risk of death/critical events (OR 0.671, CI 0.435 to 1.034, p = 0.071). Within the hypertensive cohort, however, there was a significant lower association with deaths (OR 0.664, CI 0.458 to 0.964, p = 0.031) or the combination of death/critical outcomes (OR 0.670, CI 0.495 to 0.908, p = 0.010). There was no significant association of critical/death outcomes within ACEi vs non-ACEi (OR 1.008, CI 0.822 to 1.235, p = 0.941) and ARB vs non-ARB (OR 0.946, CI 0.735 to 1.218, p = 0.668). Summary This is the largest meta-analysis including critical events and mortality data on patients prescribed ACEi/ARB and found evidence of beneficial effects of chronic ACEi/ARB use especially in hypertensive cohort with COVID-19. As such, we would strongly encourage patients to continue with RAAS inhibitor pharmacotherapy during the COVID-19 pandemic. Electronic supplementary material The online version of this article (10.1007/s11883-020-00880-6) contains supplementary material, which is available to authorized users.