Wild-type (senile) amyloidosis medical therapy: Difference between revisions
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==Overview== | ==Overview== | ||
There is no treatment for wild-type (senile) amyloidosis; the mainstay of [[therapy]] is supportive treatment aimed at [[symptoms]] of the [[disease]]. Supportive treatment is with [[Diuretic|diuretics]], [[antiarrhythmics]] or [[pacemaker]] implantation, [[Anticoagulant|anticoagulation]] where [[supraventricular arrhythmias]] are present, and an avoidance of [[digoxin]] and [[Calcium channel blocker|calcium channel blockers]]. [[Antihypertensives]] are usually poorly tolerated as these patients can be profoundly [[Hypotension|hypotensive]]. [[Pharmacological|Pharmacologic]] [[Therapy|therapies]] aimed at stabilizing the [[transthyretin]] [[molecule]] and thus preventing [[amyloid]] formation are being actively investigated. | |||
==Medical Therapy== | ==Medical Therapy== | ||
* There is no treatment for wild-type amyloidosis; the mainstay of therapy is supportive treatment aimed at symptoms of the disease. | * There is no treatment for wild-type (senile) amyloidosis; the mainstay of [[therapy]] is supportive treatment aimed at [[symptoms]] of the [[disease]]. | ||
*Since wild-type amyloidosis commonly presents with symptoms of right-sided heart failure and carpal tunnel syndrome, treating amyloid deposits in the heart is important. | *Since wild-type (senile) amyloidosis commonly presents with [[Symptom|symptoms]] of [[right-sided heart failure]] and [[carpal tunnel syndrome]], treating [[amyloid]] deposits in the [[heart]] is important.<ref name="pmid18382889">{{cite journal| author=Tanskanen M, Peuralinna T, Polvikoski T, Notkola IL, Sulkava R, Hardy J et al.| title=Senile systemic amyloidosis affects 25% of the very aged and associates with genetic variation in alpha2-macroglobulin and tau: a population-based autopsy study. | journal=Ann Med | year= 2008 | volume= 40 | issue= 3 | pages= 232-9 | pmid=18382889 | doi=10.1080/07853890701842988 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18382889 }}</ref> | ||
* Supportive treatment is with diuretics, antiarrhythmics or pacemaker implantation, anticoagulation where supraventricular arrhythmias are present, and an avoidance of digoxin and calcium channel blockers. | * Supportive treatment is with [[Diuretic|diuretics]], [[antiarrhythmics]] or [[pacemaker]] implantation, [[Anticoagulant|anticoagulation]] where [[supraventricular arrhythmias]] are present, and an avoidance of [[digoxin]] and [[Calcium channel blocker|calcium channel blockers]]. | ||
* Antihypertensives are usually poorly tolerated as these patients can be profoundly hypotensive | *[[Antihypertensives]] are usually poorly tolerated as these patients can be profoundly [[Hypotension|hypotensive]]. | ||
*For some patients with severe wild-type (senile) | *For some [[Patient|patients]] with severe wild-type (senile) amyloidosis, [[heart transplant]] may be the best option. | ||
* | *[[Pharmacological|Pharmacologic]] [[Therapy|therapies]] aimed at stabilizing the [[transthyretin]] [[molecule]] and thus preventing [[amyloid]] formation are being actively investigated. | ||
*In patients with transthyretin amyloid cardiomyopathy, Tafamidis was associated with reductions in all-cause mortality and cardiovascular-related hospitalizations and reduced the decline in functional capacity and quality of life as compared with placebo.<ref name="pmid30145929">{{cite journal| author=Maurer MS, Schwartz JH, Gundapaneni B, Elliott PM, Merlini G, Waddington-Cruz M et al.| title=Tafamidis Treatment for Patients with Transthyretin Amyloid Cardiomyopathy. | journal=N Engl J Med | year= 2018 | volume= 379 | issue= 11 | pages= 1007-1016 | pmid=30145929 | doi=10.1056/NEJMoa1805689 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30145929 }}</ref> | *In [[Patient|patients]] with transthyretin amyloid cardiomyopathy, Tafamidis was associated with reductions in all-cause mortality and [[cardiovascular]]-related [[hospitalizations]] and reduced the decline in functional capacity and quality of life as compared with [[placebo]].<ref name="pmid30145929">{{cite journal| author=Maurer MS, Schwartz JH, Gundapaneni B, Elliott PM, Merlini G, Waddington-Cruz M et al.| title=Tafamidis Treatment for Patients with Transthyretin Amyloid Cardiomyopathy. | journal=N Engl J Med | year= 2018 | volume= 379 | issue= 11 | pages= 1007-1016 | pmid=30145929 | doi=10.1056/NEJMoa1805689 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30145929 }}</ref> | ||
==References== | ==References== | ||
Latest revision as of 20:02, 19 December 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Roukoz A. Karam, M.D.[2]
Overview
There is no treatment for wild-type (senile) amyloidosis; the mainstay of therapy is supportive treatment aimed at symptoms of the disease. Supportive treatment is with diuretics, antiarrhythmics or pacemaker implantation, anticoagulation where supraventricular arrhythmias are present, and an avoidance of digoxin and calcium channel blockers. Antihypertensives are usually poorly tolerated as these patients can be profoundly hypotensive. Pharmacologic therapies aimed at stabilizing the transthyretin molecule and thus preventing amyloid formation are being actively investigated.
Medical Therapy
- There is no treatment for wild-type (senile) amyloidosis; the mainstay of therapy is supportive treatment aimed at symptoms of the disease.
- Since wild-type (senile) amyloidosis commonly presents with symptoms of right-sided heart failure and carpal tunnel syndrome, treating amyloid deposits in the heart is important.[1]
- Supportive treatment is with diuretics, antiarrhythmics or pacemaker implantation, anticoagulation where supraventricular arrhythmias are present, and an avoidance of digoxin and calcium channel blockers.
- Antihypertensives are usually poorly tolerated as these patients can be profoundly hypotensive.
- For some patients with severe wild-type (senile) amyloidosis, heart transplant may be the best option.
- Pharmacologic therapies aimed at stabilizing the transthyretin molecule and thus preventing amyloid formation are being actively investigated.
- In patients with transthyretin amyloid cardiomyopathy, Tafamidis was associated with reductions in all-cause mortality and cardiovascular-related hospitalizations and reduced the decline in functional capacity and quality of life as compared with placebo.[2]
References
- ↑ Tanskanen M, Peuralinna T, Polvikoski T, Notkola IL, Sulkava R, Hardy J; et al. (2008). "Senile systemic amyloidosis affects 25% of the very aged and associates with genetic variation in alpha2-macroglobulin and tau: a population-based autopsy study". Ann Med. 40 (3): 232–9. doi:10.1080/07853890701842988. PMID 18382889.
- ↑ Maurer MS, Schwartz JH, Gundapaneni B, Elliott PM, Merlini G, Waddington-Cruz M; et al. (2018). "Tafamidis Treatment for Patients with Transthyretin Amyloid Cardiomyopathy". N Engl J Med. 379 (11): 1007–1016. doi:10.1056/NEJMoa1805689. PMID 30145929.