Essen Amyloidosis Registry (EAR)

The Essen Amyloidosis Registry (EAR) is a prospective, observational registry designed to collect comprehensive clinical data on patients diagnosed with systemic amyloidosis. The registry aims to improve the understanding of disease progression, diagnostic pathways, and treatment outcomes. The registry is hosted at the University Hospital Essen and follows patients longitudinally. Inclusion is open to all patients with suspected or confirmed amyloidosis who provide informed consent.

Study Overview

• Status: Recruiting
• Conditions: Amyloidosis Cardiac; Amyloidosis; Systemic

Detailed Description

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.

• Study Type: Observational
• Enrollment (Estimated): 400

Contacts and Locations

• Study Contact: Name: Lars Michel, MD; Phone Number: +49 201 72384841; Email: lars.michel@uk-essen.de

Study Locations

• Germany
  • NRW
    • Essen, NRW, Germany, 45147
      • Recruiting
      • University Hospital Essen
      • Contact: Lars Michel, MD; Phone Number: +49 201 72384841; Email: lars.michel@uk-essen.de

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Adult; Older Adult
• Accepts Healthy Volunteers: Yes

• Sampling Method: Probability Sample

Study Population

All patients with suspected or confirmed amyloidosis treated at the University Hospital Duisburg-Essen are screened for inclusion. A particular focus of the registry lies on amyloid cardiomyopathy and amyloid polyneuropathy.

Description

Inclusion Criteria:

• Suspected or confirmed amyloidosis (any)
• Written informed consent to participate in the study
• Age 18 years and above

Exclusion Criteria:

• Age < 18 years
• Lack of written informed consent

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Overall mortality or unplanned hospitalization for heart failure	Overall mortality or unplanned hospitalization for heart failure is defined as the primary endpoint. The time to the primary endpoint will be measured in days starting from the day of first diagnosis of cardiac amyloidosis.	24 months

Collaborators and Investigators

• Sponsor: University Hospital, Essen

Study record dates

Study Major Dates

• Study Start (Actual): May 8, 2024
• Primary Completion (Estimated): January 1, 2029
• Study Completion (Estimated): July 1, 2029

Study Registration Dates

• First Submitted: March 14, 2025
• First Submitted That Met QC Criteria: March 14, 2025
• First Posted (Actual): March 20, 2025

Study Record Updates

• Last Update Posted (Actual): August 21, 2025
• Last Update Submitted That Met QC Criteria: August 15, 2025
• Last Verified: March 1, 2025

More Information

Terms related to this study

• Keywords: Amyloidosis; Cardiomyopathy; Neuropathy; Transthyretin; Light chain amyloidosis
• Additional Relevant MeSH Terms: Metabolic Diseases; Proteostasis Deficiencies; Amyloidosis
• Other Study ID Numbers: EAR-WDAZ1

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED
• IPD Plan Description: The study is planned as a monocentric registry but is open to requests of scientific cooperation.

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No