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.
Department of Nephrology, Ankara University School of Medicine, Ibni Sina Hospital, Ankara, 06100, Turkey.
Banu
B
Aktas Yilmaz
Nursen
N
Düzgün
Türkan
T
Mete
Levent
L
Yazicioglu
Müyesser
M
Sayki
Arzu
A
Ensari
Sehsuvar
S
Ertürk
D058186:Acute Kidney Injury / Q000209:etiology; D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D002815:Chordae Tendineae; D017809:Fatal Outcome; D005260:Female; D006333:Heart Failure / Q000209:etiology; D006341:Heart Rupture / Q000209:etiology; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000473:pathology; D008875:Middle Aged; D008944:Mitral Valve Insufficiency / Q000150:complications / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D009102:Multiple Organ Failure / Q000209:etiology* / Q000473:pathology; D009404:Nephrotic Syndrome / Q000150:complications / Q000209:etiology* / Q000473:pathology; D012772:Shock, Septic / Q000209:etiology* / Q000473:pathology