Background/Purpose: Recently there is increasing number of reports pointing out the efficacy of anti-interleukin 1(anti-IL1)therapy to control AA amyloidosis secondary to autoinflammatory diseases. Here we report our experience in IL-1 blockade in patients with AA amyloidosis secondary to FMF.

Methods:  Twenty three FMF patients withhistologically proven secondary AA amyloidosistreated with anti-IL1 agents (canacinumab and anakinra). Creatinine, creatine clearance, 24-hour urine protein, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)parameters were measured before and throughout the treatment period in order to evaluate the response. One patient on anakinra for less than a month, therefore the renal functions were not evaluated for her. Patients were closely monitored and adverse events were also reported.

Results: Twenty three (12 female, 11male) patients with AA amyloidosis secondary to FMF who were also on colchicine (mean dose: 1.86±0.58mg/day) were started on anti-IL1 agents (11 on canakinumab, 16 on anakinra).Four patients using Canakinumab were previously on Anakinra. The mean age was 42.5±10.82 years, while the mean duration of FMF was 24.3±8.2 years. The mean follow-up period on anti-IL1 was 14.9±11.5 months (14.5±13.5months on anakinra; 15.45±9.43 months on canakinumab). Fifteen patients are still on anti-IL-1 therapy (9 on anakinra, 6 on canakinumab). Renal functions remained stable after therapy for 9 out of 15 patients (24-hour urine protein from 5754,6±7787.6mg/dlto 4205.286±5717.645 mg/dl, creatinine clearance from 63.41±25.36 to 71.08±31.66 and creatinine from 1.44±0.46 to 1.62±0.69), while CRP (from 21.34±26.40 mg/L to 18.94±32.74 mg/L) and ESR (from 67.44±34.12 mm/h to 34.5±29.16 mm/h) decreased.Renal functions and acute phase reactants improved with therapy in 6 patients (24-hour urine protein from 5153.33±6432.11 mg/dlto1051.733±4571.181 mg/dl;creatinine clearance from 100,76±54.03 to 81.68±40.23 and creatinine from 1.16±0.61 to 1.11±0.60), CRPfrom 22.31±24.66 mg/Lto 2.82±10.65 mg/L; ESR from 64.16±29.73 mm/hto 13.00±14.52 mm/h).Among patients remained stable during anti-IL-1 therapy, 2 were on hemodialysis. In one patient with renal transplantation, amyloidosis was evident in the transplanted kidney and anakinra was introduced and she remained stable.Global patient assessment score of the whole group decreased significantly (from 7.04±3.07 to 1.6±1.94) with IL-1 blockade. Among patients on anakinra, in 5 patients therapy was terminated due to increased proteinuria in 3 and allergic reaction in 2. One patient recieving anakinra with end-stage renal insufficiency died in another center. Treatment was stopped in 5 patients on canakinumab because of increased proteinuria in 3,lichen planus in 1. One patient was lost to follow up despite improvement who was admitted back to the clinic 7 months later with lung adenocarcinoma. A switch from anakinra to canakinumab was made in 4 patients due to allegic reaction in 1 and non-response in 3.

Conclusion: Anti-IL1 therapy can improve or stabilize renal functions in patients with AA amyloidosis secondary to FMF.The efficacy and safety of anti-IL1 therapy in this group of patients in the long-term needs further research.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/anti-interleukin-1-therapy-in-fmf-amyloidosis-a-single-center-experience/