Publications by authors named "Allan L Klein"

A 69-year-old man presented with corticosteroid-dependent colchicine-resistant chronic recurrent pericarditis initially thought to be idiopathic in etiology. Transitioning from anakinra to rilonacept revealed rheumatoid arthritis as the underlying cause. Anakinra demonstrated superior efficacy in managing both pericarditis and rheumatoid arthritis symptoms.

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Article Synopsis
  • Recurrent pericarditis is challenging for both patients and healthcare providers due to its significant impact on health and substantial treatment costs.
  • New research since the 2015 European Society of Cardiology Guidelines has improved our understanding of the causes, imaging techniques, and treatment options available for this condition.
  • The review emphasizes the importance of imaging-guided therapy and interleukin-1 inhibitors, which have changed how recurrent pericarditis is managed, although more studies are needed to refine diagnosis and treatment timing.
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A 65-year-old woman with a history of idiopathic pericarditis presented with chronic recurrent pericarditis. Because of the inability to taper off anakinra without recurrent flares, she transitioned to rilonacept, which led to symptom abatement. Her positive response to rilonacept therapy correlated with an improvement in inflammatory changes noted on cardiac magnetic resonance imaging.

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Importance: Pericarditis accounts for up to 5% of emergency department visits for nonischemic chest pain in North America and Western Europe. With appropriate treatment, 70% to 85% of these patients have a benign course. In acute pericarditis, the development of constrictive pericarditis (<0.

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Article Synopsis
  • Recurrent pericarditis (RP) is a challenging condition with no established model for predicting patient outcomes, despite prior studies on clinical remission.
  • A risk stratification model was created using data from 365 patients to predict long-term outcomes, focusing on clinical remission defined as stopping all medications with symptoms resolved.
  • The model identified key factors, achieved a C-index of 0.800 for prediction accuracy, and effectively categorized patients into high, intermediate, and low-risk groups, improving individualized treatment decisions for RP.
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Background: Malignant pericardial effusion (Eff) is often asymptomatic and has an unknown prevalence, due to its occult presentation. The condition often is identified postmortem on autopsy, and it is associated with a poor prognosis. Given the late presentation of malignant pericardial Effs, a minimal volume of literature has examined the epidemiology, clinical characteristics, and outcomes of these complex patients.

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Aims: In the phase 3 trial, RHAPSODY, rilonacept effectively resolved active pericarditis recurrences, and long-term treatment led to sustained pericarditis recurrence risk reduction. Prior analysis suggested association between higher late gadolinium enhancement (LGE) at baseline and more rapid recurrence upon rilonacept suspension after 12 weeks of treatment. This subgroup analysis assessed the utility of longitudinal serial cardiac magnetic resonance (CMR) imaging for tracking clinical improvement and predicting post-treatment-cessation outcomes to help guide clinical decision making.

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Pericardial diseases have gained renewed clinical interest, leading to a renaissance in the field. There have been many recent advances in pericardial diseases in both multimodality cardiac imaging of diagnoses, such as recurrent, transient constrictive and effusive-constrictive pericarditis, and targeted therapeutics, especially anti-interleukin (IL)-1 agents that affect the inflammasome as part of autoinflammatory pathophysiology. There remains a large educational gap for clinicians, leading to variability in evaluation and management of these patients.

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Article Synopsis
  • Recent advances in understanding pericardial diseases and the use of multimodal imaging have significantly improved the diagnosis and treatment approaches.
  • Pericardiectomy is advised for serious cases like chronic constrictive pericarditis and requires a team of specialists for the best patient outcomes.
  • Performing radical pericardiectomy at high-volume medical centers with skilled staff enhances the likelihood of a successful surgery and recovery.
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Background: Rilonacept inhibits the interleukin-1 pathway, and extended treatment in patients with recurrent pericarditis (RP) reduced recurrence risk by 98% in the phase 3 trial, RHAPSODY long-term extension (LTE). Severe acute respiratory syndrome (SARS)-CoV-2 vaccination and/or infection may trigger pericarditis recurrence, and in clinical practice, it is unknown whether to continue rilonacept during SARS-CoV-2 infection. This post-hoc analysis of the RHAPSODY LTE aimed to inform rilonacept management in RP patients vaccinated against SARS-CoV-2 or who contract COVID-19.

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Background: Echocardiography is widely used to evaluate left ventricular (LV) diastolic function in patients suspected of heart failure. For patients in sinus rhythm, a combination of several echocardiographic parameters can differentiate between normal and elevated LV filling pressure with good accuracy. However, there is no established echocardiographic approach for the evaluation of LV filling pressure in patients with atrial fibrillation.

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Introduction: Despite the growing use of immune checkpoint inhibitors (ICI) in cancer treatment, data regarding ICI-associated pericardial disease are primarily derived from case reports and case series. ICI related pericardial disease can be difficult to diagnose and is associated with significant morbidity. We conducted a systematic review to further characterize the epidemiology, clinical presentation, and outcomes of this patient population.

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In patients with cardiac amyloidosis, pericardial involvement is common, with up to half of patients presenting with pericardial effusions. The pathophysiological mechanisms of pericardial pathology in cardiac amyloidosis include chronic elevations in right-sided filling pressures, myocardial and pericardial inflammation due to cytotoxic effects of amyloid deposits, and renal involvement with subsequent uremia and hypoalbuminemia. The pericardial effusions are typically small; however, several cases of life-threatening cardiac tamponade with hemorrhagic effusions have been described as a presenting clinical scenario.

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Aim: Anakinra, an anti IL-1 agent targeting IL-1 alfa and beta, is available for the treatment of recurrent pericarditis in cases with corticosteroid dependence and colchicine resistance after failure of conventional therapies. However, it is unclear if the combination with colchicine, a non-specific inhibitor of the inflammasome targeting the same inflammatory pathway of IL-1, could provide additional benefit to prevent further recurrences. The aim of the present observational study is to assess whether the addition of colchicine on top of anakinra could prolong the time to first recurrence and prevent recurrences better than anakinra alone.

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A 63-year-old male patient presented with chest pain and signs of volume overload. His work-up revealed a diagnosis of transient effusive constrictive pericarditis of idiopathic etiology. Despite treatment with optimal medical therapy, he continued to experience persistent symptoms eventually requiring radical pericardiectomy.

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Pericarditis in pregnancy is uncommon, and there is a paucity of data regarding the safety and efficacy of conventional therapy. We describe a complex case of recurrent pericarditis in the setting of pregnancy and newly diagnosed systemic lupus erythematosus and discuss the challenges in managing this subset of patients.

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A 40-year-old woman presented with recurrent pericarditis and pericardial effusion while receiving treatment with all-trans retinoic acid and arsenic trioxide for recently diagnosed acute promyelocytic leukemia. She was successfully treated with the interleukin-1 inhibitor rilonacept after experiencing multiple recurrences with triple therapy with aspirin, colchicine, and steroids. ().

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Derangements in the innate and adaptive immune responses observed in systemic inflammatory syndromes contributes to unique elevated atherosclerotic risk and incident cardiovascular disease. Novel multimodality imaging techniques may improve diagnostic precision for the screening and monitoring of disease activity. The integrated application of these technologies lead to earlier diagnosis and noninvasive monitoring of cardiac involvement in systemic inflammatory diseases that will aid in preclinical studies, enhance patient selection, and provide surrogate endpoints in clinical trials, thereby improving clinical outcomes.

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Article Synopsis
  • Tafamidis has been shown to improve survival rates in patients with transthyretin amyloid cardiomyopathy (ATTR-CM), but its specific impact on cardiac function was unclear, prompting further investigation.
  • The study analyzed data from the ATTR-ACT clinical trial, involving 436 patients with ATTR-CM, to compare cardiac function measures over 30 months between those receiving tafamidis and a placebo.
  • Results indicated that patients treated with tafamidis experienced less deterioration in key cardiac function metrics, suggesting a potential benefit of the drug in managing cardiac issues related to ATTR-CM.
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Background: There is currently no thromboembolic risk stratification tool for amyloid cardiomyopathy (ACM) and the current survival staging systems for ACM have only modest discriminatory ability.

Objectives: This study aims to evaluate the prognostic value of left atrial (LA) strain to predict incident thrombotic event (TE) and improve survival staging systems in ACM.

Methods: The authors identified patients with light chain (AL) or transthyretin (ATTR) ACM and no history of atrial fibrillation (AF) at diagnosis.

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