Wild-type (senile) amyloidosis natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]

Overview

Wild-type (senile) amyloidosis, as the name suggests, is a disease of the elderly. The clinical picture of the disease corresponds to the type of organ or organ system involved. It most commonly affects the heart and hence, clinical features pertaining to cardiac pathologies, dominate the clinical course of the disease. If left untreated, wild-type (senile) amyloidosis can lead to heart failure with reduced ejection fraction (HFrEF) and eventually death. Wild-type (senile) amyloidosis is most commonly complicated by heart failure with reduced ejection fraction (HFrEF). The median duration of survival after diagnosis is 75 months.

Natural History, Complications, and Prognosis

Natural History

In amyloidosis, insoluble fibrils of amyloid are deposited in the organs, causing organ dysfunction and eventually death.^[1]
Wild-type (senile) amyloidosis, as the name suggests, is a disease of the elderly.
The clinical picture of the disease corresponds to the type of organ or organ system involved.
It most commonly affects the heart and hence, clinical features pertaining to cardiac pathologies, dominate the clinical course of the disease.
The deposition of transthyretin (TTR) in the heart causes it to start functionally failing.^[2]
The constellation of signs and symptoms of a TTR affected heart can mimic heart failure due to old age, and can thus mask the systemic involvement of wild-type (senile) amyloidosis.
If left untreated, wild-type (senile) amyloidosis can lead to heart failure with reduced ejection fraction (HFrEF) and eventually death.^[3]

Complications

Wild-type (senile) amyloidosis is most commonly complicated by heart failure with reduced ejection fraction (HFrEF).^[3]
Other complications include:

Prognosis

In comparison with AL amyloidosis, the severity of heart failure in wild-type (senile) amyloidosis is less.^[5]
The median duration of survival after diagnosis is 75 months.^[6]

References

↑ Baker KR, Rice L (2012). "The amyloidoses: clinical features, diagnosis and treatment". Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.
↑ Belinda Ng, Lawreen H. Connors, Ravin Davidoff, Martha Skinner & Rodney H. Falk (2005). "Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis". Archives of internal medicine. 165 (12): 1425–1429. doi:10.1001/archinte.165.12.1425. PMID 15983293. Unknown parameter |month= ignored (help)
↑ ^3.0 ^3.1 Belinda Ng, Lawreen H. Connors, Ravin Davidoff, Martha Skinner & Rodney H. Falk (2005). "Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis". Archives of internal medicine. 165 (12): 1425–1429. doi:10.1001/archinte.165.12.1425. PMID 15983293. Unknown parameter |month= ignored (help)
↑ Claudio Rapezzi, Giampaolo Merlini, Candida C. Quarta, Letizia Riva, Simone Longhi, Ornella Leone, Fabrizio Salvi, Paolo Ciliberti, Francesca Pastorelli, Elena Biagini, Fabio Coccolo, Robin M. T. Cooke, Letizia Bacchi-Reggiani, Diego Sangiorgi, Alessandra Ferlini, Michele Cavo, Elena Zamagni, Maria Luisa Fonte, Giovanni Palladini, Francesco Salinaro, Francesco Musca, Laura Obici, Angelo Branzi & Stefano Perlini (2009). "Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types". Circulation. 120 (13): 1203–1212. doi:10.1161/CIRCULATIONAHA.108.843334. PMID 19752327. Unknown parameter |month= ignored (help)
↑ Belinda Ng, Lawreen H. Connors, Ravin Davidoff, Martha Skinner & Rodney H. Falk (2005). "Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis". Archives of internal medicine. 165 (12): 1425–1429. doi:10.1001/archinte.165.12.1425. PMID 15983293. Unknown parameter |month= ignored (help)
↑ Belinda Ng, Lawreen H. Connors, Ravin Davidoff, Martha Skinner & Rodney H. Falk (2005). "Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis". Archives of internal medicine. 165 (12): 1425–1429. doi:10.1001/archinte.165.12.1425. PMID 15983293. Unknown parameter |month= ignored (help)

[pmid23227278-1] Baker KR, Rice L (2012). "The amyloidoses: clinical features, diagnosis and treatment". Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.

[2] Belinda Ng, Lawreen H. Connors, Ravin Davidoff, Martha Skinner & Rodney H. Falk (2005). "Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis". Archives of internal medicine. 165 (12): 1425–1429. doi:10.1001/archinte.165.12.1425. PMID 15983293. Unknown parameter |month= ignored (help)

[:0-3] 3.0 ^3.1 Belinda Ng, Lawreen H. Connors, Ravin Davidoff, Martha Skinner & Rodney H. Falk (2005). "Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis". Archives of internal medicine. 165 (12): 1425–1429. doi:10.1001/archinte.165.12.1425. PMID 15983293. Unknown parameter |month= ignored (help)

[4] Claudio Rapezzi, Giampaolo Merlini, Candida C. Quarta, Letizia Riva, Simone Longhi, Ornella Leone, Fabrizio Salvi, Paolo Ciliberti, Francesca Pastorelli, Elena Biagini, Fabio Coccolo, Robin M. T. Cooke, Letizia Bacchi-Reggiani, Diego Sangiorgi, Alessandra Ferlini, Michele Cavo, Elena Zamagni, Maria Luisa Fonte, Giovanni Palladini, Francesco Salinaro, Francesco Musca, Laura Obici, Angelo Branzi & Stefano Perlini (2009). "Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types". Circulation. 120 (13): 1203–1212. doi:10.1161/CIRCULATIONAHA.108.843334. PMID 19752327. Unknown parameter |month= ignored (help)

[5] Belinda Ng, Lawreen H. Connors, Ravin Davidoff, Martha Skinner & Rodney H. Falk (2005). "Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis". Archives of internal medicine. 165 (12): 1425–1429. doi:10.1001/archinte.165.12.1425. PMID 15983293. Unknown parameter |month= ignored (help)

[6] Belinda Ng, Lawreen H. Connors, Ravin Davidoff, Martha Skinner & Rodney H. Falk (2005). "Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis". Archives of internal medicine. 165 (12): 1425–1429. doi:10.1001/archinte.165.12.1425. PMID 15983293. Unknown parameter |month= ignored (help)

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