scholarly journals [18F]-Florbetaben PET/CT for Differential Diagnosis Among Cardiac Immunoglobulin Light Chain, Transthyretin Amyloidosis, and Mimicking Conditions

Author(s):  
Dario Genovesi ◽  
Giuseppe Vergaro ◽  
Assuero Giorgetti ◽  
Paolo Marzullo ◽  
Michele Scipioni ◽  
...  
2021 ◽  
Vol 23 (7) ◽  
Author(s):  
Thomas Hellmut Schindler ◽  
Monica Sharma ◽  
Ines Valenta ◽  
Alessio Imperiale ◽  
Vasken Dilsizian

2019 ◽  
Vol 9 (6) ◽  
Author(s):  
Victoria E Thomas ◽  
Justin Smith ◽  
Merrill D Benson ◽  
Noel R Dasgupta

Amyloidosis is a group of diseases characterized by extracellular deposition of amyloid fibril complexes. Fibril deposition results in organ dysfunction and possible failure. Amyloidosis is regarded as a rare disease, but in general is underdiagnosed. The two main types of systemic amyloidosis are immunoglobulin light chain and transthyretin amyloidosis. The increased availability of noninvasive cardiac imaging, genetic testing and improved laboratory assays and protein identification methods have led to increased diagnosis. However, in many cases, the diagnosis is not made until the patient develops organ impairment. Earlier diagnosis is required to prevent irreversible organ failure. Novel treatments for immunoglobulin light chain and transthyretin amyloidosis that halt disease progression, prolong and increase quality of life have recently become available.


2016 ◽  
Vol 41 (2) ◽  
pp. e115-e117 ◽  
Author(s):  
Stephen M. Broski ◽  
Robert J. Spinner ◽  
Benjamin M. Howe ◽  
Angela Dispenzieri ◽  
Geoffrey B. Johnson

2019 ◽  
Vol 27 (17) ◽  
pp. 1806-1815 ◽  
Author(s):  
Giuseppe Vergaro ◽  
Alberto Aimo ◽  
Andrea Barison ◽  
Dario Genovesi ◽  
Gabriele Buda ◽  
...  

Cardiac involvement in systemic amyloidosis, due either to immunoglobulin light-chain or transthyretin amyloidosis, influences clinical presentation and is a strong predictor of unfavourable outcome. Until recently considered as a rare, incurable disease, cardiac amyloidosis, is still mis/underdiagnosed, although treatments effective in improving patient survival are now available for both subtypes, including chemotherapy regimens for immunoglobulin light-chain amyloidosis and tetramer stabiliser for transthyretin amyloidosis. Achieving a timely diagnosis allows initiating life-saving therapies and requires the early recognition of clinical, laboratory and imaging signs of cardiac involvement, some of them may be apparent well before the disease becomes clinically manifest. Given the systemic nature of amyloidosis, a close interaction among experts in multiple specialties is also required, including cardiologists, nephrologists, haematologists, neurologists, radiologists, nuclear medicine specialists and internists. As an increased awareness about disease presentation is required to ameliorate diagnostic performance, we aim to provide the clinician with a guide to the screening and early diagnosis of cardiac amyloidosis, and to review the clinical, biohumoral and instrumental ‘red flags’ that should raise the suspicion of cardiac amyloidosis.


2021 ◽  
Vol 2021 (2) ◽  
Author(s):  
Feihong Ding ◽  
Yun Li ◽  
Shailesh Balasubramanian ◽  
Subha Ghosh ◽  
Jason N Valent ◽  
...  

ABSTRACT Amyloidosis is a heterogeneous group of diseases characterized by the extracellular deposition of misfolded proteins that can affect either systemically or locally confined to one system. Pulmonary amyloidosis is rare and can be classified into three forms according to the anatomic site of involvement: nodular pulmonary amyloidosis, tracheobronchial amyloidosis and diffuse alveolar-septal amyloidosis. The former two usually represent localized amyloid disease and the latter represents systemic disease. Typically lung parenchymal and tracheobronchial amyloidosis do not present together in localized forms of pulmonary amyloidosis. Here we report a unique case of localized pulmonary immunoglobulin light-chain amyloidosis, manifested as both parenchymal nodules and tracheobronchial amyloid deposition.


Author(s):  
Haruki Koike ◽  
Naohiro Mouri ◽  
Yuki Fukami ◽  
Masahiro Iijima ◽  
Koji Matsuo ◽  
...  

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