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Causes and diagnosis of AA amyloidosis and relation to rheumatic diseases

Author
Peter D Gorevic, MD
Section Editor
Helen J Lachmann, MA, MB, BChir, MD, FRCP, FRCPath
Deputy Editor
Paul L Romain, MD

INTRODUCTION

AA amyloidosis (previously known as secondary [AA] amyloidosis) is a disorder characterized by the extracellular tissue deposition of fibrils that are composed of fragments of and/or intact serum amyloid apolipoprotein A (SAA), a hepatic acute phase reactant. (See "Pathogenesis of AA amyloidosis".)

AA amyloidosis may complicate any chronic inflammatory condition, including rheumatoid arthritis (RA), juvenile idiopathic arthritis, ankylosing spondylitis (AS), inflammatory bowel disease, familial periodic fever syndromes, chronic infections, and certain neoplasms (table 1).

The major causes and approach to diagnosis of AA amyloidosis are presented here. The clinical manifestations and treatment of AA amyloidosis and the musculoskeletal and renal manifestations of amyloid diseases are discussed separately. (See "Overview of amyloidosis", section on 'Clinical manifestations' and "Treatment of secondary (AA) amyloidosis" and "Musculoskeletal manifestations of amyloidosis" and "Renal amyloidosis".)

CAUSES AND RELATION TO RHEUMATIC DISEASES

Multiple chronic inflammatory conditions, among them rheumatologic, autoinflammatory, chronic infectious, and other disorders, have been associated with the development of AA amyloid. The most common organ system involved in AA amyloidosis is the kidney, although other organ systems are often also affected. (See 'Clinical manifestations' below and "Overview of amyloidosis", section on 'Clinical manifestations'.)

Epidemiology — The disorders most often identified as underlying AA amyloidosis have varied over time and geographically, reflecting the prevalent chronic inflammatory conditions. Inflammatory arthritides have become the most common causes, although chronic infections, particularly tuberculosis and osteomyelitis, were historically the predominant causes. With widespread availability of highly effective antimicrobial agents, the infectious causes have become less common in much of the world, and it is hoped that therapeutic advances in the treatment of rheumatologic and other inflammatory disorders with highly effective biologic agents will result in fewer patients developing amyloidosis [1].

               
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Literature review current through: Oct 2017. | This topic last updated: Nov 20, 2017.
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