Amyloidosis, Transthyretin Amyloidosis
Conditions
Brief summary
This is a single center prospective study evaluating 124I-evuzumitide in patients with systemic amyloidosis. The purpose of this study is to 1) Establish the diagnostic accuracy of 124I-evuzumitide in cardiac amyloidosis 2) Evaluate extracardiac uptake 3) identify and characterize the distribution and uptake of 124I-evuzumitide in patients with transthyretin amyloid cardiomyopathy (ATTR-CM) and 4) Correlate the uptake with the structure and function of different organs, including the heart. To achieve these goals, eligible patients will undergo primarily hybrid positron emission tomography and magnetic resonance imaging (PET/MRI). In a subgroup of patients who are unable to undergo PET/MR, computed tomography will be used instead of MRI (i.e. PET/CT). In a subgroup of patients, repeat imaging with the same modality will be done at a interval of 6-12 months. Clinically available data (demographics, phenotype, imaging, laboratory) will also be collected to characterize the disease in each patient.
Interventions
DIAGNOSTIC_TEST124I-Evuzamitide
Amyloid reactive protein used as imaging agent to detect systemic amyloidosis
Sponsors
Oregon Health and Science University
Attralus, Inc.
Bayer
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
DIAGNOSTIC
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
40 Years to 90 Years
Healthy volunteers
No
Inclusion criteria
1. Subjects will at least have one of the following conditions: systemic amyloidosis with known organ involvement, carrier of a known pathogenic mutation in the transthyretin gene, multiple myeloma, and monoclonal gammopathy of undetermined significance 2. Patient willing to consent for the study and undergo the study procedures.
Exclusion criteria
1. Has severe claustrophobia or any medical condition that would prevent completion of the imaging protocol 2. Has a known allergy to potassium iodide treatment or to gadolinium. 3. Patients on dialysis or those with eGFR \<30 cc/min/1.73 m2 will be excluded from undergoing gadolinium-enhanced cardiac MRI. 4. Has received heparin or heparin analogs (e.g., enoxaparin, dalteparin, fondaparinux) within 7 days prior to 124I-Evuzumitide administration
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| I-124 evuzamitide diagnostic accuracy | At baseline scan | To evaluate the efficacy of I-124 evuzamitide for diagnosing cardiac amyloidosis based on visual scan interpretation, compared to SoC site diagnosis. We will assess: * The sensitivity of PET/MRI with 124I-evuzamitide PET/MRI for the diagnosis of cardiac amyloidosis. * The specificity of PET/MRI with 124I-evuzamitide for the diagnosis of cardiac amyloidosis. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Quantitative measures | At baseline scan | To evaluate the efficacy of I-124 evuzamitide for diagnosing cardiac amyloidosis based on quantitative scan interpretation, compared to SoC site diagnosis. We will assess the sensitivity and specificity of various quantitative measures, and evaluate inter- and intra-reader agreement. |
| Correlation between clinical cardiac biomarkers (i.e. stage of amyloidosis) and myocardial 124I-Evuzamitide uptake | At baseline scan | Pearson Correlation Coefficient of stage of amyloidosis (Mayo Staging System) and SUV (124I-Evuzamitide) |
| Correlation between Kansas City Cardiomyopathy Questionnaire overall summary score (KCCQ-OS) and myocardial 124I-Evuzamitide | During baseline scan and follow up scan | — |
| Correlation between New York Heart Association (NYHA) class and 124I-Evuzamitide uptake | During baseline scan and follow up scan | — |
| Correlation between liver and spleen ECV and their respective 124I-Evuzamitide uptake | During baseline scan and follow up scan | — |
| Proportion of patients with ATTR-CM and MGUS or smoldering myeloma who are correctly classified as ATTR-CM based on 124I-Evuzamitide distribution and uptake | During baseline scan | — |
| To evaluate the safety of a single intravenous administration of I-124 evuzamitide. | 7 days from baseline scan | Incidence of related treatment-emergent (S)AEs from time of study drug administration to Day 7 |
Countries
United States
Contacts
CONTACTAhmad Masri, MD
Outcome results
None listed