Causes and diagnosis of secondary (AA) amyloidosis and relation to rheumatic diseases
- Peter D Gorevic, MD
Peter D Gorevic, MD
- Professor of Medicine, Rheumatology
- Mount Sinai School of Medicine
Secondary (AA) amyloidosis is a disorder characterized by the extracellular tissue deposition of fibrils that are composed of fragments of serum amyloid A (SAA) protein, an acute phase reactant. (See "Pathogenesis of AA amyloidosis".)
AA amyloidosis may complicate a number of chronic inflammatory conditions, including rheumatoid arthritis, juvenile chronic polyarthritis, ankylosing spondylitis, inflammatory bowel disease, familial periodic fever syndromes, chronic infections, and certain neoplasms.
The major causes and approach to diagnosis of AA amyloidosis, as well as the rheumatic manifestations of other forms of amyloidosis, are presented here. The clinical manifestations and treatment of AA amyloidosis are discussed separately. (See "Overview of amyloidosis", section on 'Clinical manifestations' and "Treatment of secondary (AA) amyloidosis".)
CAUSES AND RELATION TO RHEUMATIC DISEASES
The overall autopsy incidence of secondary (AA) amyloidosis in western nations ranges from 0.5 to 0.86 percent [1,2]. A decreasing incidence of AA amyloidosis has also been reflected in national surveys from some western countries [3-5]. The following frequency of underlying disorders has been found in large series of patients with AA amyloidosis from two large centers in the United States [1,6,7]:
●Rheumatoid arthritis (RA) or juvenile idiopathic arthritis (JIA) – 12 to 21 percent
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- CAUSES AND RELATION TO RHEUMATIC DISEASES
- Rheumatoid arthritis
- Juvenile idiopathic arthritis
- Ankylosing spondylitis
- Systemic lupus erythematosus
- Other rheumatic diseases
- Hereditary AA amyloidosis
- Idiopathic AA amyloidosis
- CLINICAL MANIFESTATIONS
- Laboratory testing
- SAP scintigraphy
- DIFFERENTIAL DIAGNOSIS
- SUMMARY AND RECOMMENDATIONS