Abstract
BackgroundOngoing inflammatory episodes of familial Mediterranean fever (FMF) disease can cause damage in nearly all organ systems. Colchicine and interleukin1β blocking agents are successfully used to control the disease activity [1]. Although the effect of IL-1 blockers are known for controlling disease activity and amyloidosis [2], it is unclear whether they prevent organ damageObjectivesIn our study, we assessed the organ damage in patients with FMF treated with colchicine and IL-1 antagonists (IL-1A). It was evaluated whether new damage occurred after IL-1 antagonist treatment.MethodsA total of 111 patients fulfilling Tel-Hashomer criteria and treated with IL-1A due to colchicine resistance were included in the study. All patients were also treated with colchicine with a maximum-tolerable dose. Patients were grouped according to their recent damage status (no damage, pre-existing damage, and damage developed under IL-1A treatment). The degree of damage was determined using Autoinflammatory Disease Damage Index (ADDI) and modified form of ADDI (mADDI) [3, 4].Results44 patients (42,3%) had damage according to the modified ADDI (mADDI) index; three patients experienced new damage under IL-1 antagonist treatment while four patients showed progression of damage and mADDI score.In patients with a positive mADDI score, the most common damage was amyloidosis (n=28, 63%), the second most frequent was musculoskeletal findings (N=14, 31%), and the third was infertility (N=2, 0,04%). The most common domains of FMF-related damage with IL-1 antagonist treatment were musculoskeletal (n=4), renal (n=2) and reproductive system (n=2)ConclusionOur study was the first study to evaluate the progression of damage in patients with FMF and treated- with IL-1 antagonists. Although it is known that IL-1A is effective in colchicine-resistant patients, physicians should be aware that damage can still develop under IL-1A treatment.
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