Wild-type (senile) amyloidosis medical therapy: Difference between revisions
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==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. | ||
* | *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. | ||
*For some patients with severe wild-type (senile) ATTR amyloidosis, a heart transplant may be the best option. | * 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) ATTR amyloidosis, a heart transplant may be the best option. | |||
*In ATTRwt amyloidosis, therapy is supportive, but both for this disease and for ATTR, pharmacologic therapies aimed at stabilizing the transthyretin molecule and thus preventing amyloid formation are being actively investigated. | *In ATTRwt amyloidosis, therapy is supportive, but both for this disease and for ATTR, 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.<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== | ||
Revision as of 14:17, 30 October 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
Medical Therapy
- There is no treatment for wild-type 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.
- 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) ATTR amyloidosis, a heart transplant may be the best option.
- In ATTRwt amyloidosis, therapy is supportive, but both for this disease and for ATTR, 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.[1]
References
- ↑ 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.