Objective: To study amyloidosis as a cause of death along with associated factors and frequency of pre-mortem diagnosis in patients with rheumatoid arthritis (RA) autopsied between 1952 and 1991.
Methods: We studied causes of death in 369 consecutively autopsied RA and 370 autopsied non-RA patients of the same sex, age at death, and year of autopsy. In those RA patients who died from 1973 onwards, we were also able to analyse clinical data: pre-mortem diagnosis of amyloidosis, clinical features of RA, and treatment.
Results: Based on autopsy, amyloidosis was determined as a cause of death in 9.5% of RA and in none of the non-RA patients (p<0.001). In our RA patients, we detected no trend in deaths from amyloidosis between 1952 and 1991. The RA patients dying of amyloidosis died younger than those dying of other causes (p=0.001). During the course of the disease, the RA patients with amyloidosis had: higher erythrocyte sedimentation rate (p=0.002), lower haemoglobin (p<0.001), more frequently proteinuria (p<0.001) and renal failure (p<0.001) than did the rest of the RA patients. Pre-mortem, amyloidosis was diagnosed by biopsy in 65% of the RA patients with amyloidosis as their cause of death.
Conclusion: Amyloidosis may be undetected during the course of RA. Thus, it should be actively searched for in the patients with long-lasting and active disease, especially, if they have proteinuria or renal failure.
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Front Med (Lausanne)
January 2025
Key Laboratory of Obstetric and Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, Department of Radiology, West China Second University Hospital, Sichuan University, Chengdu, China.
Hereditary heart disease (HHD) is a series of cardiac disorders associated with monogenic or polygenic abnormalities and is one of the leading causes of sudden death, particularly in young adults. The updated European Cardiology guideline for cardiomyopathies provides the first comprehensive summary of genotyping, imaging, and therapy recommendations for inherited cardiomyopathies, but still lacks a comprehensive discussion of research advances and future trends in genetic diagnosis and therapy of HHD. Our research aims to fill this gap.
View Article and Find Full Text PDFJ Cardiovasc Med (Hagerstown)
February 2025
Cardiology Unit, Azienda Ospedaliera Universitaria di Ferrara, Cona, Ferrara, Italy.
Introduction: Cardiac amyloidosis typically causes restrictive cardiomyopathy, in which the impairment of diastolic function is dominant. Echocardiography provides prognostic information through some important parameters: left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS). However, LVEF often remains preserved despite disease progression, and GLS is not routinely performed as it is limited by suboptimal image quality.
View Article and Find Full Text PDFPLoS One
January 2025
Phantomics, Inc., Seoul, South Korea.
Aims: T1 mapping on cardiac magnetic resonance (CMR) imaging is useful for diagnosis and prognostication in patients with light-chain cardiac amyloidosis (AL-CA). We conducted this study to evaluate the performance of T1 mapping parameters, derived from artificial intelligence (AI)-automated segmentation, for detection of cardiac amyloidosis (CA) in patients with left ventricular hypertrophy (LVH) and their prognostic values in patients with AL-CA.
Methods And Results: A total of 300 consecutive patients who underwent CMR for differential diagnosis of LVH were analyzed.
JAMA Cardiol
January 2025
National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Hospital, London, United Kingdom.
Importance: Patients with transthyretin (ATTR) cardiac amyloid infiltration are increasingly diagnosed at earlier disease stages with no heart failure (HF) symptoms and a wide range of cardiac amyloid infiltration.
Objective: To characterize the clinical phenotype and natural history of asymptomatic patients with ATTR cardiac amyloid infiltration.
Design, Setting, And Participants: This cohort study analyzed data of all patients at 12 international centers for amyloidosis from January 1, 2008, through December 31, 2023.
Amyloid
January 2025
Department of Cardiology, Complejo Hospitalario Universitario de A Coruña (CHUAC), Instituto de Investigación Biomédica de A Coruña (INIBIC), A Coruña, Spain.
Aims: To evaluate the predictive value of the Columbia score in patients with transthyretin amyloid cardiomyopathy (ATTR-CM).
Methods: Observational study based in a prospective, multi-centre registry of patients with ATTR-CM recruited between January-2018 and December-2023 in 7 Spanish hospitals. The Baseline Columbia score was correlated by means of multivariable Cox's regression with study endpoints all-cause death and all-cause death or heart failure (HF) hospitalisation.
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