Wild-type (senile) amyloidosis natural history, complications and prognosis: Difference between revisions

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{{Wild-type (senile) amyloidosis}}
{{Wild-type (senile) amyloidosis}}
{{CMG}}
{{CMG}}{{AE}}{{Sab}}


==Overview==
==Overview==
Wild-type (senile) amyloidosis, as the name suggests, is a [[disease]] of the [[Old age|elderly]]. The clinical picture of the [[disease]] corresponds to the type of [[Organ (anatomy)|organ]] or organ system involved. It most commonly affects the [[heart]] and hence, clinical features pertaining to [[Heart|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|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|heart failure with reduced ejection fraction (HFrEF)]]. The [[median]] duration of survival after [[diagnosis]] is 75 months.


==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==
Line 9: Line 10:


* In [[amyloidosis]], insoluble fibrils of [[amyloid]] are deposited in the [[Organ (anatomy)|organs]], causing organ dysfunction and eventually death.<ref name="pmid23227278">{{cite journal |vauthors=Baker KR, Rice L |title=The amyloidoses: clinical features, diagnosis and treatment |journal=Methodist Debakey Cardiovasc J |volume=8 |issue=3 |pages=3–7 |date=2012 |pmid=23227278 |pmc=3487569 |doi= |url=}}</ref>
* In [[amyloidosis]], insoluble fibrils of [[amyloid]] are deposited in the [[Organ (anatomy)|organs]], causing organ dysfunction and eventually death.<ref name="pmid23227278">{{cite journal |vauthors=Baker KR, Rice L |title=The amyloidoses: clinical features, diagnosis and treatment |journal=Methodist Debakey Cardiovasc J |volume=8 |issue=3 |pages=3–7 |date=2012 |pmid=23227278 |pmc=3487569 |doi= |url=}}</ref>
*Wild-type (senile) amyloidosis, as the name suggests, is a disease of the elderly.
*Wild-type (senile) amyloidosis, as the name suggests, is a [[disease]] of the [[Old age|elderly]].
*The clinical picture of the disease corresponds to the type of organ or organ system involved.
*The clinical picture of the [[disease]] corresponds to the type of [[Organ (anatomy)|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.
*It most commonly affects the [[heart]] and hence, clinical features pertaining to [[Heart|cardiac]] pathologies, dominate the clinical course of the [[disease]].
*The deposition of transthyretin (TTR) in the heart causes it to start functionally failing.
*The deposition of [[Transthyretin|transthyretin (TTR)]] in the [[heart]] causes it to start [[Heart failure|functionally failing]].<ref>{{Cite journal
*The constellation of signs and symptoms of a TTR affected heart can mimic heart failure due to old age and can thus mask wild-type (senile) amyloidosis.
| author = [[Belinda Ng]], [[Lawreen H. Connors]], [[Ravin Davidoff]], [[Martha Skinner]] & [[Rodney H. Falk]]
| title = Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis
| journal = [[Archives of internal medicine]]
| volume = 165
| issue = 12
| pages = 1425–1429
| year = 2005
| month = June
| doi = 10.1001/archinte.165.12.1425
| pmid = 15983293
}}</ref>
*The constellation of [[Medical sign|signs]] and [[Symptom|symptoms]] of a [[Transthyretin|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|heart failure with reduced ejection fraction (HFrEF)]] and eventually death.<ref name=":0">{{Cite journal
| author = [[Belinda Ng]], [[Lawreen H. Connors]], [[Ravin Davidoff]], [[Martha Skinner]] & [[Rodney H. Falk]]
| title = Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis
| journal = [[Archives of internal medicine]]
| volume = 165
| issue = 12
| pages = 1425–1429
| year = 2005
| month = June
| doi = 10.1001/archinte.165.12.1425
| pmid = 15983293
}}</ref>
*
*


===Complications===
===Complications===
* Wild-type (senile) amyloidosis is most commonly complicated by [[Heart failure with reduced ejection fraction|heart failure with reduced ejection fraction (HFrEF)]].<ref name=":0" />
* Other [[Complication (medicine)|complications]] include:
**[[Left bundle branch block|Left bundle branch block (LBBB)]]<ref>{{Cite journal
| author = [[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]]
| title = Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types
| journal = [[Circulation]]
| volume = 120
| issue = 13
| pages = 1203–1212
| year = 2009
| month = September
| doi = 10.1161/CIRCULATIONAHA.108.843334
| pmid = 19752327
}}</ref>
** [[Peripheral neuropathy]]
** [[Tendon]] [[rupture]]


===Prognosis===
===Prognosis===
*In comparison with [[Primary amyloidosis|AL amyloidosis]], the severity of [[heart failure]] in wild-type (senile) amyloidosis is less.<ref>{{Cite journal
| author = [[Belinda Ng]], [[Lawreen H. Connors]], [[Ravin Davidoff]], [[Martha Skinner]] & [[Rodney H. Falk]]
| title = Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis
| journal = [[Archives of internal medicine]]
| volume = 165
| issue = 12
| pages = 1425–1429
| year = 2005
| month = June
| doi = 10.1001/archinte.165.12.1425
| pmid = 15983293
}}</ref>
*The [[median]] duration of survival after [[diagnosis]] is 75 months.<ref>{{Cite journal
| author = [[Belinda Ng]], [[Lawreen H. Connors]], [[Ravin Davidoff]], [[Martha Skinner]] & [[Rodney H. Falk]]
| title = Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis
| journal = [[Archives of internal medicine]]
| volume = 165
| issue = 12
| pages = 1425–1429
| year = 2005
| month = June
| doi = 10.1001/archinte.165.12.1425
| pmid = 15983293
}}</ref>


==References==
==References==

Latest revision as of 19:24, 17 December 2019

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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

Complications

Prognosis

References

  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)
  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)