the diagnostic value of hybrid pet/mr for systemic amyloidosis

Conditions: Systemic Amyloidosis; PET/MR

Summary: 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.

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.

Eligibility criteria:
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