ESPN 51th Annual Meeting

ESPN 2018


 
TRANSPLANTATION IN FAMILIAL MEDITERRANEAN FEVER RELATED AMYLOIDOSIS WITHIN THE ERA OF ANTI IL-1 THERAPY
ZEYNEP BIRSIN ÖZÇAKAR 1 KENAN KEVEN 2 NILGÜN ÇAKAR 1 FATOŞ YALÇINKAYA 1

1- ANKARA UNIVERSITY MEDICAL FACULTY, PEDIATRIC NEPHROLOGY, ANKARA, TURKEY
2- ANKARA UNIVERSITY MEDICAL FACULTY, NEPHROLOGY, ANKARA, TURKEY
 
Introduction:

Longterm outcomes of patients with familial Mediterranean fever (FMF) related amyloidosis who had received kidney transplantation were controversial. In recent years, interleukin (IL)-1 inhibitors have been used in the treatment of FMF patients who had developed amyloidosis. Very little data exists about the use of these agents in renal transplant recipients. The aim of this study was to present clinical findings and treatment responses of renal transplant patients with FMF related amyloidosis, who were treated with anti IL-1 therapies.

Material and methods:

All patients with FMF related amyloidosis who received a kidney transplant in our center between the years 2008-2018 and who were treated with IL-1 inhibitors were included in this report.

Results:

Among 68 transplantations 5 received a kidney due to FMF related amyloidosis. Mean age at the diagnosis of amyloidosis was 10.3 ± 2.9 years (range 6 -13.5) and at the time of transplantation was 17.3±1.8 (range 15.5-19.5). Three patients who were on anti IL-1 therapy, due to ongoing attacks and amyloidosis before transplantation, were proceeded with this therapy within the first week of transplantation. The two other patients were not on anti-IL-1 therapy prior to transplantation and these agents were started at the 10th and 32nd month of transplantation. Patients were followed on anti IL-1 therapy with a minimum of 18 with the longest follow-up of 81 months. They had no or mild few attacks during anti IL-1 therapy and acute phase reactants were completely normal beyond the periods of infection and attacks. Three of the 5 patients required hospitalization due to infection only once. Tonsillitis, pneumonia, fungal otitis media are the causes of these hospitalizations. None of the patients had proteinuria in the last visit.

Conclusions:

Anti IL-1 therapies seem to be emerging effective and safe treatment options in patients with FMF related amyloidosis after renal transplantation.