4.0 Article

Secondary, AA, Amyloidosis

Journal

RHEUMATIC DISEASE CLINICS OF NORTH AMERICA
Volume 44, Issue 4, Pages 585-+

Publisher

W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.rdc.2018.06.004

Keywords

Systemic amyloidosis; AA amyloidosis; Serum amyloid A; SAA1 gene; Congo red staining; SAP scintigraphy; Rheumatoid arthritis; Autoinflammatory syndrome

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Secondary, AA, amyloidosis is a rare systemic complication that can develop in any long-term inflammatory disorder and is characterized by the extracellular deposition of fibrils derived from serum amyloid A (SAA) protein. SAA is an acute-phase reactant synthetized largely by hepatocytes under the transcriptional regulation of proinflammatory cytokines. The kidney is the major involved organ, with proteinuria as the first clinical manifestation; renal biopsy is the most common diagnostic investigation. Targeted anti-inflammatory treatment promotes normalization of circulating SAA levels preventing amyloid deposition and renal damage. Novel therapies aimed at promoting clearance of existing amyloid deposits soon may be an effective treatment approach.

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