Heredofamilial Amyloidosis.

A variety of familial forms of amyloidosis have been described. Most of them are rare and occur in limited geographic areas. The most common and best studied is an autosomal recessive

condition called familial Mediterranean fever.[172] This is a febrile disorder of unknown cause characterized by attacks of fever accompanied by inflammation of serosal surfaces, including

peritoneum, pleura, and synovial membrane. This disorder is encountered largely in individuals of Armenian, Sephardic Jewish, and Arabic origins. It is associated with widespread tissue

involvement indistinguishable from reactive systemic amyloidosis. The amyloid fibril proteins are made up of AA proteins, suggesting that this form of amyloidosis is related to the

recurrent bouts of inflammation that characterize this disease. The gene for familial Mediterranean fever has been cloned, and its product is called pyrin (for its relation to fever). Although

its exact function is not known, it has been suggested that pyrin is responsible for regulating acute inflammation, presumably by inhibiting the function of neutrophils.[173] The relationship

of this mutation to the disease is not understood.

In contrast to familial Mediterranean fever, a group of autosomal dominant familial disorders is characterized by deposition of amyloid predominantly in the nerves—peripheral and

autonomic. These familial amyloidotic polyneuropathies have been described in different parts of the world. As mentioned previously, in all of these genetic disorders, the fibrils are made

up of mutant transthyretins (ATTR).

Localized Amyloidosis.

Sometimes, amyloid deposits are limited to a single organ or tissue without involvement of any other site in the body. The deposits may produce grossly detectable nodular masses or be

evident only on microscopic examination. Nodular (tumor-forming) deposits of amyloid are most often encountered in the lung, larynx, skin, urinary bladder, tongue, and the region about

the eye. Frequently, there are infiltrates of lymphocytes and plasma cells in the periphery of these amyloid masses, raising the question of whether the mononuclear infiltrate is a response

to the deposition of amyloid or instead is responsible for it. At least in some cases, the amyloid consists of AL protein and may therefore represent a localized form of immunocyte-derived

amyloid.

Endocrine Amyloid.

Microscopic deposits of localized amyloid may be found in certain endocrine tumors, such as

medullary carcinoma of the thyroid gland, islet tumors of the pancreas, pheochromocytomas, and undifferentiated carcinomas of the stomach, and in the islets of Langerhans in patients

with type II diabetes mellitus. In these settings, the amyloidogenic proteins seem to be derived either from polypeptide hormones (e.g., medullary carcinoma) or from unique proteins (e.g.,

islet amyloid polypeptide).

Amyloid of Aging.

Several well-documented forms of amyloid deposition occur with aging.[174] Senile systemic amyloidosis refers to the systemic deposition of amyloid in elderly patients (usually in their

seventies and eighties). Because of the dominant involvement and related dysfunction of the heart, this form was previously called senile cardiac amyloidosis. Those who are symptomatic

present with a restrictive cardiomyopathy and arrhythmias. The amyloid in this form is composed of the normal TTR molecule. In addition to the sporadic senile systemic amyloidosis,

another form, affecting predominantly the heart, that results from the deposition of a mutant form of TTR has also been recognized. Approximately 4% of the black population in the

United States is a carrier of the mutant allele, and cardiomyopathy has been identified in both homozygous and heterozygous patients. The precise prevalance of patients with this mutation

who develop clinically manifest cardiac disease is not known.

Pathogenesis.

Amyloidosis results from abnormal folding of proteins, which are deposited as fibrils in extracellular tissues and disrupt normal function. Misfolded proteins are often unstable and selfassociate,

ultimately leading to the formation of oligomers and fibrils that are deposited in tissues. The reason diverse conditions are associated with amyloidosis may be that each of these

conditions results in excessive production of proteins that are prone to misfolding. The proteins that form amyloid fall into two general categories: (1) normal proteins that have an inherent

tendency to fold improperly, associate and form fibrils, and do so when they are produced in

Figure 6-54Proposed schema of the pathogenesis of the major forms of amyloid fibrils.

Figure 6-55Amyloidosis of the kidney. The glomerular architecture is almost totally obliterated by the massive accumulation of amyloid.

Figure 6-56Cardiac amyloidosis. The atrophic myocardial fibers are separated by structureless, pink-staining amyloid (arrows).

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