The Diagnostic Value of Hybrid PET/MR for Systemic Amyloidosis

Systemic amyloidosis is a multi-system disease caused by extracellular deposition of insoluble amyloid fibrils in various tissues and organs, leading to progressive organ dysfunction. The clinical manifestations of different types of amyloidosis are complex and diverse, and the prognosis is very poor. Early detection and classification of amyloid deposition is becoming increasingly important. However, conventional imaging techniques including ultrasound and magnetic resonance are not sensitive or specific. Endocardial biopsy is the gold standard for the diagnosis of cardiac amyloidosis, but it is an invasive procedure with a clinical complication rate of 6%.

Positron emission tomography (PET) provides a valuable tool for diagnosing systemic amyloidosis. Recently, amyloid PET imaging agents (11C-PIB or 18F-florbetapir) have been shown to be effective as novel positron tracers to detect potential amyloid deposition in some small sample studies. The investigators will use the most advanced imaging equipment, integrated PET/MR with amyloid PET imaging agents(11C-PIB or 18F-florbetapir) to image patients suspected or confirmed systemic amyloidosis, the aim is to explore the value of hybrid PET/MR for systemic amyloidosis.

Study Overview

• Status: Recruiting
• Conditions: PET/MR; Systemic Amyloidosis
• Intervention / Treatment: Diagnostic test: 11C-PIB or 18F-florbetapir PET/MR before biopsy and treatment; Diagnostic test: 11C-PIB or 18F-florbetapir PET/MR after treatment

Detailed Description

Systemic amyloidosis is a multi-system disease caused by extracellular deposition of insoluble amyloid fibrils in various tissues and organs, leading to progressive organ dysfunction. The clinical manifestations of different types of amyloidosis are complex and diverse, and the prognosis is very poor. Early detection and classification of amyloid deposition is becoming increasingly important. However, conventional imaging techniques including ultrasound and magnetic resonance are not sensitive or specific. Endocardial biopsy is the gold standard for the diagnosis of cardiac amyloidosis, but it is an invasive procedure with a clinical complication rate of 6%.

Positron emission tomography (PET) provides a valuable tool for diagnosing systemic amyloidosis. Recently, amyloid PET imaging agents (11C-PIB or 18F-florbetapir) have been shown to be effective as novel positron tracers to detect potential amyloid deposition in multiple organs in some small sample studies. The investigators will use the most advanced imaging equipment, integrated PET/MR with amyloid PET imaging agents(11C-PIB or 18F-florbetapir) to image patients suspected or confirmed systemic amyloidosis, the aim is to explore the value of hybrid PET/MR for systemic amyloidosis.

For patients suspected of or diagnosed with systemic amyloidosis, the investigators aim to evaluate the roles of hybrid PET/MR in differential diagnosis, detecting the deposition of amyloid in various tissues and organs of the body, guiding biopsy, and determining treatment plan prior to treatment; for the patients with a history of systemic amyloidosis, the aim is to evaluate the value of hybrid PET/MR for treatment response assessment.

• Study Type: Observational
• Enrollment (Anticipated): 30

Contacts and Locations

Study Locations

• China
  • Hubei
    • Wuhan, Hubei, China, 430022
      • Recruiting
      • China, Hubei Province

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (ADULT, OLDER_ADULT)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Central China

Description

Inclusion Criteria:

Patient with Monoclonal Ganunopathy, adds one of the following criteria:

• Histologically confirmed Amyloidosis of any organ.
• Average left ventricular thickness of the echocardiogram is more than 11 mm without uncontrolled high blood pressure.
• 12-lead ECG shows unexplained low voltage <0.5 mV.

Exclusion Criteria:

• Patient can not lie flat
• NYHA Level 4 Heart Failure
• Patient is pregnant or nursing
• Patient is allergic to amyloid PET imaging agents
• Patient with acute systemic diseases and electrolyte disorders
• Patient with severe claustrophobia or unstable vital sigh
• Other serious comorbidities evaluated by primary investigator

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
11C-PIB or 18F-florbetapir PET/MR; Patients suspected of or diagnosed with systemic amyloidosis will be scanned by 11C-PIB or 18F-florbetapir PET/MR twice. One is before biopsy and treatment, and the other is after at least half a year of treatment.	Diagnostic test: 11C-PIB or 18F-florbetapir PET/MR before biopsy and treatment; 10-20 mCi 11C-PIB or 5-10 mCi 18F-florbetapir will be injected intravenously prior to imaging.; Diagnostic test: 11C-PIB or 18F-florbetapir PET/MR after treatment; 10-20 mCi 11C-PIB or 5-10 mCi 18F-florbetapir will be injected intravenously prior to imaging.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Sensitivity and specificity per patient analysis	For patient without any treatment, detection and initial diagnosis, results of 11C-PiB or 18F-florbetapir PET/MR will be compared to histopathological, clinical, laboratory, radiological evidence and follow-up result.	up to 2 years

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Sensitivity and specificity per organ analysis	For patient without any treatment, detection and initial diagnosis, results of 11C-PiB or 18F-florbetapir PET/MR will be compared to histopathological, clinical, laboratory, radiological evidence and follow-up result.	up to 2 years
Change after treatment	For patient after treatment, change of PET/MR scan and clinical/radiological/histopathological indices.	up to 2 years
Correlation with severity	Correlation of 11C-PiB or 18F-florbetapir uptake with clinical/radiological/histopathological indices of amyloidosis severity.	up to 2 years

Collaborators and Investigators

• Sponsor: Wuhan Union Hospital, China
• Investigators: Principal Investigator: Xiaoli Lan, MD, PhD, Wuhan Union Hospital, China

Study record dates

Study Major Dates

• Study Start (ACTUAL): March 11, 2019
• Primary Completion (ANTICIPATED): December 31, 2023
• Study Completion (ANTICIPATED): December 31, 2023

Study Registration Dates

• First Submitted: June 30, 2019
• First Submitted That Met QC Criteria: June 30, 2019
• First Posted (ACTUAL): July 5, 2019

Study Record Updates

• Last Update Posted (ESTIMATE): February 14, 2023
• Last Update Submitted That Met QC Criteria: February 13, 2023
• Last Verified: February 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Metabolic Diseases; Proteostasis Deficiencies; Amyloidosis
• Other Study ID Numbers: XLan-S895

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No