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Treatment of secondary (AA) amyloidosis

Peter D Gorevic, MD
Section Editor
Peter H Schur, MD
Deputy Editor
Paul L Romain, MD


Secondary (AA) amyloidosis is a disorder characterized by the extracellular tissue deposition of fibrils composed of fragments of serum amyloid A protein (SAA), an acute phase reactant. AA amyloidosis can complicate a number of chronic inflammatory conditions, including rheumatoid arthritis (RA), juvenile idiopathic arthritis (JIA), and ankylosing spondylitis [1,2]. (See "Pathogenesis of secondary (AA) amyloidosis".)


If untreated, secondary (AA) amyloidosis is a serious disease with a significant mortality due to end-stage renal disease, infection, heart failure, bowel perforation, or gastrointestinal bleeding [3-5]. Patients with persistently high circulating levels of serum amyloid A protein (SAA) are at particular risk to develop these complications of disease [6,7]. A progressive improvement in survival rates has been reported among patients with AA amyloidosis, likely reflecting, in part, improved treatment strategies for associated inflammatory disorders, as well as earlier detection [3].

In a review of 374 cases of AA amyloidosis, for example, mean survival from diagnosis was 133 months, with older age, reduced serum albumin concentration, end-stage renal failure at baseline, and the degree by which the SAA concentration was elevated during follow-up being of poorer prognostic significance [3].

Importance of control of the underlying disease — Successful treatment of the underlying inflammatory process (as with surgical resection of the focus of infection or tumor), such as cytotoxic agents and/or biological agents in rheumatoid arthritis (RA) or antibiotics with chronic infection, can lead to stabilization of or improvement in renal function, to reduction in protein excretion, and to partial resolution of amyloid deposits (as assessed in some studies by scintigraphy with radiolabeled serum amyloid P component [SAP]) [8-14]. This was illustrated in a report of 80 patients with AA amyloid, mostly due to juvenile idiopathic arthritis or to RA, who were prospectively followed for a median of four years; in this series, the systemic amyloid load was assessed by yearly SAP scintigraphy [15]. The following findings were noted:

Forty-two patients had median serum SAA concentration within the reference range (<10 mg/L); amyloid deposits regressed in 25 and stabilized in 14. Among patients with renal disease at baseline, proteinuria typically fell, while the serum creatinine concentration was either stable or improved. Two patients who required dialysis remained on dialysis.


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Literature review current through: Sep 2016. | This topic last updated: Jan 2, 2016.
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