The Essen Amyloidosis Registry (EAR) is a single-center, prospective, observational
registry designed to systematically collect clinical, laboratory, imaging, and outcome
data from patients with suspected or confirmed systemic amyloidosis. The registry aims to
improve the understanding of disease characteristics, diagnostic pathways, and treatment
outcomes. While cardiac amyloidosis remains a main focus, EAR includes all forms of
systemic amyloidosis, such as light-chain (AL) amyloidosis, transthyretin amyloidosis
(ATTR, both hereditary and wild-type), and rarer subtypes.
Data collected include demographic information, medical history, comorbidities,
diagnostic findings, treatment strategies, and longitudinal follow-up data on disease
progression and therapy response. Standardized assessments include serial laboratory
tests, cardiac and other imaging modalities (e.g., echocardiography, cardiac MRI,
scintigraphy), and functional status evaluations. Routine quality-of-life assessments, as
part of standard clinical practice, are also documented. Patients will not be required to
complete additional study-specific questionnaires.
The registry further integrates a biobank component, in which biological samples are
collected for biomarker analysis. Given the current limitations of available
diagnostics-such as insufficient sensitivity for early disease detection and a lack of
robust markers for therapy monitoring-these biospecimens may help identify new prognostic
and predictive biomarkers.
EAR enables the analysis of risk factors, disease progression, and long-term outcomes
based on real-world clinical data. The registry also serves as an internal quality
control tool, ensuring standardized data collection and treatment monitoring. Patients
remain under regular clinical follow-up, with routine evaluations every 3 to 6 months,
depending on their disease stage and treatment regimen. These visits typically include
resting ECGs, blood tests, imaging studies, and device checks (for patients with
pacemakers or defibrillators).
The Essen Amyloidosis Registry aims to create a comprehensive dataset that can be
utilized for future research projects. Sub-projects addressing specific scientific
questions will be submitted as separate ethics applications. The study follows standard
clinical care guidelines, with examinations performed according to established Standard
Operating Procedures (SOPs) for amyloidosis management.