Background: Autoinflammatory diseases (AIDs) are a heterogeneous group of rare genetically determined conditions. Biologics (B) are used, especially inhibitors of interleukin 1 (iIL-1), in the treatment of the most common monogenic AIDs (mAID) – FMF, TRAPS, HIDS/MKD, CAPS. IIL-1 has shown high efficiency and led to significant progress in the treatment of these patients (pts). Currently, iIL1 are the first-line drugs for mAIDs therapy, primarily CAPS. In pts with systemic juvenile idiopathic arthritis (sJIA) IL-1 inhibitors are also successfully used in the first or second line of therapy along with IL-6 inhibitors.

Objectives: To evaluate in the real clinical practice the frequency of prescribing, efficacy and safety of IL-1 therapy in pts with mAIDS and sJIA according to the Federal Rheumatology Center.

Methods: A retrospective study from 2013 to 2021 included 66 pts who were prescribed iIL-1. Among them 45 pts with mAIDS: FMF - 8, CAPS - 25, TRAPS - 10, HIDS/MKD – 2, 21 pts had sJIA. Canakinumab (CAN) was administered subcutaneously at the dose of 2-5mg/ kg or 150mg every 4-8 weeks, anakinra (ANA) - subcutaneously at the dose of 1-5mg/ kg or 100mg daily.

Results: Among pts with mAIDS 45 received iIL-1. The age of pts ranged from 1.5 to 44 years, the median (Ме) age was 9.5[6.9;17] years. Female pts predominated (60%). The median age at onset was 0.5 [0;4] years (0 - 35 y). The median duration of the disease was 7.5 [3.9;15.1] years (3 months to 44 years). 35 pts received CAN, 10 - ANA. Both drugs showed significant positive dynamics with a complete response in 40 (88.9%), a partial response in 5 (11.1%), mainly due to serious neurological and cognitive impairments in 2 pts with CINCA/NOMID, sensorineural hearing loss in 3 adult pts with MWS, as well as amyloidosis in 1. In 7 pts with CAPS who received ANA as first biologic treatment, after achieving a reliable positive response, switching to CAN was performed while maintaining full efficacy. It was possible to discontinue the glucocorticoids (GC) in all pts. In 2 pts with CINCA/NOMID (1), MWS (1) due to insufficient efficiency the interval between injections of CAN was reduced from 8 to 4 weeks. The duration of use of CAN in pts with mAIDS ranged from 6 months to 12 years, ANA – 2 months to 12 years, 13 pts (28.9%) have been receiving therapy for 5 years or more. The age of pts with sJIA ranged from 3 to 17 years, Me 8,15 [5,3; 12,7] years. The age of the onset varied from 4 months to 12 years, Me 3,1 [1,8; 5,7] years. Female pts predominated (61,9%). 20 pts received CAN (18 after secondary inefficiency/infusion reaction of tocilizumab (TCZ), 2 had previous experience of 3 B: TCZ-etanercept-adalimumab, TCZ-abatacept-adalimumab). 1 patient received ANA. The duration of CAN treatment was from 5 months to 4.5 years. Among pts with sJIA who received CAN, secondary inefficiency with discontinuation was observed in 6 (28,6%) (10-25 months after treatment initiation), and in 1patient who received anakinra for 8 months. The tolerability of therapy was generally good. We have not observed any SAE in the treatment of iIL-1. All pts are continue taking medications.

Conclusion: Among the B iIL-1 are used in most pts with mAIDS, these are currently first-line drugs, especially for CAPS and TRAPS. They have high efficiency and good tolerance for long-term use. While among pts with sJIA there is a high frequency of secondary inefficiency and discontinuation, which, however, may be due to the late timing of iIL-1 treatment initiation. As a test of administration, and base on the concept of window of opportunity in treatment sJIA pts it is possible to use short-acting iIL-1 (ANA) with switch to CAN as a drug with a prolonged duration of action.

Disclosure of Interests: Svetlana Salugina Speakers bureau: Novartis, Sobi, Maria Kaleda Speakers bureau: Pfizer, Roche, Novartis, Evgeny Fedorov Speakers bureau: Novartis, Sobi, MEDAC, Irina Nikishina Speakers bureau: Pfizer, MSD, Roche, Novartis, Sobi, Anna Torgashina Speakers bureau: Novartis