secondary amyloidosis

amyloidosis /am·y·loi·do·sis/ (am″ĭ-loi-do´sis) a group of conditions characterized by the accumulation of insoluble fibrillar proteins (amyloid) in various organs and tissues such that vital function is compromised. The associated disease states may be inflammatory, hereditary, or neoplastic, and deposition can be local, generalized, or systemic. The most widely used classification is based on the chemistry of the amyloid fibrils and includes primary (AL), secondary (AA), and familial forms.

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AA amyloidosis  secondary a.

AL amyloidosis  primary a.

ATTR amyloidosis  the most common form of familial amyloidosis (q.v.), associated with mutations of the gene encoding transthyretin.

familial amyloidosis  any inherited form of amyloidosis; usually used to denote any of various systemic autosomal dominant disorders of amyloid deposition that involve the nervous system. The most common form, ATTR amyloidosis, is associated with mutations of the transthyretin protein, but rare mutations of other proteins can also be a cause. The term can also, less commonly, be said to include several types of secondary amyloidosis in which the deposition is associated with an inherited disease (e.g., familial Mediterranean fever). Subclassifications are based on clinical presentation and biochemical composition of the fibrils deposited; originally they were distinguished on the basis of kinship. See also familial amyloid polyneuropathy, under polyneuropathy.

hemodialysis-associated amyloidosis  that occurring in patients on long-term hemodialysis, caused by the deposition of beta2-microglobulin, which cannot be removed from the blood by hemodialysis, in the joints, synovial membranes, and tendon sheaths. Manifestations include carpal tunnel syndrome and arthritis.

hereditary amyloidosis  familial a.

primary amyloidosis  a systemic form in which the deposited fibrillar material is AL amyloid; it may be due to either aberrant synthesis or processing of immunoglobulin light chains. It is associated with tumors or dyscrasias of immunoglobulin-producing plasma cells and involves some combination of the skin and subcutaneous tissue, nerves, liver, spleen, heart, kidney, intestine, and tongue. Called also AL a..

reactive systemic amyloidosis  secondary a.

renal amyloidosis  amyloid deposits in the kidneys; in the primary type the fibrils are mainly AL amyloid, and in secondary types they are AA amyloid. Secondary types may accompany inflammatory disorders, chronic infectious diseases, or neoplastic diseases.

secondary amyloidosis  that in which AA amyloid is deposited, and which occurs secondary to a chronic inflammatory condition, either infectious or noninfectious. It usually involves the liver, spleen, and kidneys.

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secondary amyloidosis

n.

Amyloidosis that occurs in association with another chronic disease and is characterized by the deposition of amyloid in fibrous connective tissue, especially in the spleen, liver, kidneys, adrenal glands, and arterioles.

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secondary amyloidosis.

See amyloidosis.

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amyloidosis (am´iloidō´sis),

n a condition in which amyloid, a glycoprotein, is deposited intercellularly in tissues and organs. Four types of amyloidosis are recognized, two of which, primary amyloidosis and amyloid tumor, frequently produce nodules in the tongue and gingiva.

Amyloidosis.

amyloidosis, primary,

n a type occurring without a known predisposing cause. Amyloid deposits are found in the tongue, lips, skeletal muscles, and other mesodermal structures. The disease may be manifested by polyneuropathy, purpura, hepatosplenomegaly, heart failure, and the nephrotic syndrome.

amyloidosis, secondary,

n a type occurring secondary to chronic diseases such as tuberculosis, leprosy, rheumatoid arthritis, multiple myeloma, and prolonged bacterial infections. Amyloid deposits are found in parenchymal organs. The disease is usually manifested by proteinuria and hepatosplenomegaly.

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amyloidosis

the deposition in various tissues of amyloid. This protein is almost insoluble and once it infiltrates the tissues they become waxy and nonfunctioning. Systemic amyloidosis may be immunocytic or reactive (see below).

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cutaneous amyloidosis

multiple cutaneous, hard, painless, chronic plaques occur over the head, neck and shoulders of horses. There may be involvement of the nasal mucosa and resulting dyspnea.

familial renal amyloidosis of Shar pei dogs

manifested by episodic fever and swelling of one or both hocks which may resolve spontaneously, but is recurring. The condition is resistant to treatment and eventually there is renal and/or hepatic failure.

immunocytic amyloidosis, immunogenic amyloidosis, primary amyloidosis

amyloid produced from light chains of immunoglobulins as in plasma-cell dyscrasias. See also al protein.

reactive amyloidosis

is derived from excess serum protein SAA produced as a result of chronic antigenic stimulation. The kidney is most often affected and the amyloid is most often deposited in glomeruli but medullary deposits are seen in cats and cattle. Idiopathic amyloidosis is common in the dog and less common in cats. It is associated with chronic suppurative disease processes in cattle, antiserum production in horses, and it occurs rarely in pigs. Called also secondary alopecia.

renal amyloidosis

characterized by severe proteinuria and uremia. There is chronic diarrhea, polydipsia and anasarca. Seen particularly in dogs and cats.

secondary amyloidosis

reactive amyloidosis (above).