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

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.