Amyloidosis is a group of metabolic familial or inherited, degenerative, and infectious disease processes, characterized by the abnormal protein folding and deposition of a complex substance composed of amyloid protein and fibrils (fine fibers). Amyloid A amyloidosis (AA) is the most common form of systemic amyloidosis worldwide. It is characterized by extracellular tissue deposition of fibrils that are composed of fragments of serum amyloid A (SAA) protein, a major acute-phase reactant protein, produced mainly by liver cells. There are several types of amyloid and the classification of amyloidosis is based on which amyloid protein is involved. A particular type of amyloidosis is designated by a capital A (for amyloid) followed by an abbreviation for the fibril protein. Twenty-three different fibril proteins are described in human amyloidosis with variable clinical features.
In all forms of amyloidosis, the cell secretes the precursor protein in a soluble form that becomes insoluble at some tissue site, compromising organ function. Amyloidosis may develop in the course of a chronic inflammatory disease of either infectious or noninfectious origin, hereditary periodic fevers, neoplasms such as Hodgkin disease and renal cell carcinoma.1 Glomerular amyloidosis usually causes urinary excretion of protein. The disease is considered to be inherited in the Chinese Shar-Pei breed.2
Disease associated with amyloidosis: Rheumatoid arthritis, juvenile chronic arthritis ankylosing spondylitis, psoriasis, adult-onset Still disease, Crohn disease, leprosy, osteomyelitis, tuberculosis, chronic bronchiectasis, Castleman disease, Hodgkin disease.
Amyloidosis is considered to be inherited in the Shar Pei
- Mohammed Mubashir Ahmed, MD. Amyloidosis, AA (Inflammatory)
- Cynthia M. Khan, BA, MA and Scott Line, DVM, PdD, Dipl ACVB. The Merck/Merial Manual for Pet Health Home Edition. Whitehouse Station, NJ: Merck & Co., Inc., 2007: 294, 307
- Bruce A Baethge, MD. "Amyloidosis, Overview." eMedicine, August 11, 2006.