Wild-type (senile) amyloidosis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
In 1639, Nicolaus Fontanus autopsied a young man who had ascites, jaundice, liver abscess, and splenomegaly and his report has been the first description of amyloidosis. There is no significant data regarding the historical perspective of amyloidosis throughout the 18th century. Rudolph Virchow and Weber are the prominent figures with substantial work on amyloidosis during the 19th century. In 1922, Bennhold introduced Congo Red staining of amyloid that remains the gold standard for diagnosis. There is no established system for the classification of wild-type (senile) amyloidosis. Amyloid is an abnormal insoluble extracellular protein that deposits in the different tissues and causes organ dysfunction and a wide variety of clinical syndromes. Wild-type (senile) amyloidosis is a type of systemic amyloidosis as transthyretin (TTR) deposits can be found throughout the body. TTR results in pathologies due to misfolding, breaking apart, and deposition of the amyloid fibrils in healthy tissue. The condition mainly affects the heart. However, other organ systems, such as the nervous and musculoskeletal systems, can also be involved. There are no genes implicated in the causality of wild-type (senile) amyloidosis. Aging is very strongly associated with wild-type (senile) amyloidosis. Wild-type (senile) amyloidosis is caused by the folding and/breaking apart of a normal occurring protein, transthyretin (TTR). Wild-type (senile) amyloidosis can be differentiated from other conditions that present with heart failure, polyneuropathy, and organomegaly. The incidence of amyloidosis is approximately 1.2 per 100,000 individuals per year worldwide. The actual incidence of wild-type (senile) amyloidosis in particular is unknown. The mortality rate of systemic amyloidosis is approximately 100 per 100,000 deaths in developed countries. Patients with wild-type (senile) amyloidosis are almost always elderly (65 years of age or older). There is no racial predilection to wild-type (senile) amyloidosis. Men are traditionally more commonly affected by wild-type (senile) amyloidosis than women. Aging has been implicated to be a risk factor for the development of wild-type (senile) amyloidosis. There is insufficient evidence to recommend routine screening for wild-type (senile) amyloidosis.
Historical Perspective
In 1639, Nicolaus Fontanus autopsied a young man who had ascites, jaundice, liver abscess, and splenomegaly and his report has been the first description of amyloidosis. There is no significant data regarding the historical perspective of amyloidosis throughout the 18th century. Rudolph Virchow and Weber are the prominent figures with substantial work on amyloidosis during the 19th century. In 1922, Bennhold introduced Congo Red staining of amyloid that remains the gold standard for diagnosis.
Classification
There is no established system for the classification of wild-type (senile) amyloidosis.
Pathophysiology
Amyloid is an abnormal insoluble extracellular protein that deposits in the different tissues and causes organ dysfunction and a wide variety of clinical syndromes. Wild-type (senile) amyloidosis is a type of systemic amyloidosis as transthyretin (TTR) deposits can be found throughout the body. TTR results in pathologies due to misfolding, breaking apart, and deposition of the amyloid fibrils in healthy tissue. The condition mainly affects the heart. However, other organ systems, such as the nervous and musculoskeletal systems, can also be involved. There are no genes implicated in the causality of wild-type (senile) amyloidosis. Aging is very strongly associated with wild-type (senile) amyloidosis.
Causes
Wild-type (senile) amyloidosis is caused by the folding and/breaking apart of a normal occurring protein, transthyretin (TTR).
Differentiating Wild-type (senile) amyloidosis from Other Diseases
Wild-type (senile) amyloidosis can be differentiated from other conditions that present with heart failure, polyneuropathy, and organomegaly.
Epidemiology and Demographics
The incidence of amyloidosis is approximately 1.2 per 100,000 individuals per year worldwide. The actual incidence of wild-type (senile) amyloidosis in particular is unknown. The mortality rate of systemic amyloidosis is approximately 100 per 100,000 deaths in developed countries. Patients with wild-type (senile) amyloidosis are almost always elderly (65 years of age or older). There is no racial predilection to wild-type (senile) amyloidosis. Men are traditionally more commonly affected by wild-type (senile) amyloidosis than women.
Risk Factors
Aging has been implicated to be a risk factor for the development of wild-type (senile) amyloidosis.
Screening
There is insufficient evidence to recommend routine screening for wild-type (senile) amyloidosis.