Background/Purpose: A decline in the frequency of AA amyloidosis secondary to RA and infectious diseases has been reported. This is probably due to more effective treatment strategies. We had previously reported that although amyloidosis occurs in less than 0.5% of BS pts. It is one of the leading causes of death (1-3). We aimed to determine the change in the frequency of AA amyloidosis over years in BS pts in addition to elaborating on clinical characteristics and outcome.

Methods: We performed a chart review to identify all pts with amyloidosis in our BS center since1976. We noted demographic characteristics, BS manifestations, age at BS and AA amyloidosis diagnosis, treatment modalities of these pts. Our endpoints were death and end stage renal disease (ESRD) requiring renal replacement therapy. The prevalence of AA amyloidosis was calculated separately for 2 periods (pts registered between 1976-2000 and 2000-2017)

Results: Among our 9410 BS pts, 27 (0.29%) had amyloidosis. We identified 24 pts with amyloidosis among the 3820 pts in the earlier cohort and 3 additional amyloidosis among the 5590 pts in the recent cohort. The frequency of AA amyloidosis had declined from 0.62% to 0.054% in the recent cohort. M/F ratio was 22/5 and mean age at BS diagnosis was 29.5±7.4 yrs. Twenty-two (82%) of the pts with AA amyloidosis had major organ involvement (vascular inv. in 15, eye inv.in 13 and neurologic inv.in 2). AA amyloidosis was diagnosed after a mean duration of 9.8±6.7 yrs and was confirmed with renal biopsy in 14 pts and rectal biopsy in 13. Eight pts had non-nephrotic range proteinuria at amyloidosis diagnosis. After amyloidosis diagnosis, 24 pts continued their previous immunosuppressives and colchicine. Two of these 24 were on anti-TNFs at amyloidosis diagnosis. Biologics were initiated in 3 pts who were most recently diagnosed to have amyloidosis, anti-TNFs in 2 and tocilizumab in 1. Fourteen (52%) pts had died after a median follow-up of 3 (IQR:1-8.75) yrs, 3 were lost to follow-up just after amyloidosis diagnosis and 10 (37%) are still alive after a median follow up of 16 (IQR:10-23) yrs. The reasons for death were infections in 5, related to ESRD in 5, subarachnoid hemorrhage, gastric adenocarcinoma, liver cirrhosis probably associated with amyloidosis and iatrogenic bowel perforation in 1 pts each. 10 (71%) of these 14 pts had developed ESRD before their deaths. Overall, 15/27 pts developed ESRD after a median follow-up of 3.5 (IQR:1.25-6.5) yrs after amyloidosis diagnosis. 5 of them had renal transplantation, all but 1 are still alive after 3, 4, 6, and 12 yrs. The last one died 11 years after transplantation due to subarachnoid hemorrhage as explained above.

Conclusion:

AA amyloidosis appears to be a rare, but fatal complication of BS. Around 50% of pts died after a median follow-up of 3 yrs after amyloidosis. This study showed a decreasing trend of amyloidosis due to BS similar to that observed in other inflammatory and infectious causes. The shorter follow-up duration may be contributing for the lower prevalence of amyloidosis in the recent cohort.

References

1)Yurdakul S,Arthritis Rheum.1990 2)Melikoğlu M,Rheumatology (Oxford).2001 3)Kural-Seyahi E,Medicine (Baltimore).2003

« Back to 2018 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/a-declining-trend-in-frequency-of-secondary-amyloidosis-in-behcets-syndrome/