Background Recently there is increasing number of reports investigating the efficacy of anti-interleukin-1 (anti-IL1) therapy in AA-amyloidosis.
Objectives Here we report our experience in IL-1 blockade in AA amyloidosis secondary to FMF.
Methods Twenty nine FMF patients with secondary AA-amyloidosis with insufficient response to colchicine were treated with anti-IL-1 agents (canakinumab and anakinra). Creatinine (Cre), 24-hour urine protein (UP), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were measured before and throughout the treatment to evaluate the response and side effects.
Results Twenty nine (16 female, 13 male) patients with FMF-related amyloidosis were administered anti-IL1 agents (12 on canakinumab, 17 on anakinra) in addition to colchicine in 27 patients, with a mean dose of 1.4±0.6 mg/day. The mean age was 40.13±11.76, while the mean duration of FMF was 28.79±10.51 years. The mean follow-up was 13.92±11.31 months for anakinra and 11.82±9.92 months for canakinumab.
Initial Cre levels were less than 1.5 mg/dl in 13 patients (range 0.37-.5). In this subgroup proteinuria decreased significantly from 3739.87±4860.41 to 1321.45±2015.62 mg/day while Cre was stable (0.91±0.30 mg/dl to 1.04±0.39 mg/dl) and acute phase response was normalized (CRP from 6.16±7.86 mg/l to 5.20±9.64 mg/l, ESR from 27.3±18.63 to 14±8.04).In the second group there were 11 patients with initial Cre levels higher than 1.5mg/dl (range 1.73–3.76). Proteinuria decreased from 6321.66±5936.43 to 4827.55±6264.43 mg/day, Cre increased from 2.53±0.76 to 3.07±1.70mg/dl, while there was a decrease in the APR in this subgorup (CRP from 40±64.65 mg/l to 26.71±26.76 mg/l, ESR from 57.37±37.65 to 39.11±27.72).
There are two patients on hemodialysis whereas two underwent renal transplantation. Global patient assessment score of the whole group was decreased from 7.44±2.54 to 3.89±3.53 with anti-IL-1.
Anakinra was stopped in 11 and canakinumab in 3 patients, due to irresponsiveness in 8 and 2 patients respectively. Among patients in whom anakinra was terminated 9 were later treated with canakinumab. Twenty four patients are still recieving anti-IL-1treatment (16 on anakinra, 8 on canakinumab).
Conclusions Anti IL-1 treatments seems to be effective and safe in the treatment of AA amyloidosis secondary to FMF. The beneficial effect of this approach is more pronounced in patients with creatinine levels of less that 1.5 mg/dl.
Disclosure of Interest None declared
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