Clinical outcome and survival of secondary (AA) amyloidosis

OBJECTIVE: In order to predict the clinical outcome of secondary (AA) amyloidosis patients with rheumatic diseases, we studied the clinical features at presentation of AA amyloidosis. METHODS: We investigated the clinical characteristics and survival of 42 patients with biopsy-proven AA amyloidosis who were followed up in our department from 1983 to 2001. RESULTS: A presenting factor which adversely influenced clinical outcome was a raised serum creatinine concentration at the time AA amyloidosis was diagnosed. Eight of 42 patients survived for 10 years or more after the presentation of AA amyloidosis, while 24 patients had died within 10 years. At the diagnosis of AA amyloidosis, cardiac involvement was detected in 11 of 24 non-survivors, whereas it was not detected in any of the 8 long-term survivors. Estimated survival at 5 years was 31.3% in those who had cardiac involvement, and was 63.3% in those who had no cardiac involvement at the presentation of AA amyloidosis. CONCLUSION: Our results indicate that clinical outcome is related to renal function and cardiac involvement at the time AA amyloidosis is diagnosed. Amyloid-related cardiac involvement is one of the unfavorable predictive factors in AA amyloidosis patients.