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Rheumatol Int. 2008 Feb;28(4):367-70. Epub 2007 Aug 9.

AA amyloidosis associated with systemic lupus erythematosus: impact on clinical course and outcome.

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Department of Nephrology, Ankara University School of Medicine, Ibni Sina Hospital, Ankara, 06100, Turkey.


Secondary amyloidosis (AA amyloidosis) has rarely been described in patients with systemic lupus erythematosus (SLE). We, herein, present a 56-year-old female patient, who developed AA amyloidosis following a 22-year history of SLE. She developed severe mitral regurgitation complicated with chordae tendinea rupture that led to acute congestive heart failure and went on a mitral valve replacement, where no flare symptoms of SLE were present. Three months after the operation, she presented with a nephrotic-range proteinuria, acute renal failure, and severe sepsis. She was found to have new vegetations on replaced valve and multi-organ failure caused her death. Re-evaluation of the excised mitral valve revealed AA amyloid deposition. Post-mortem biopsies from the kidney and bone marrow also revealed secondary amyloidosis.

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