Abstract

BackgroundRheumatoid factor-positive subtype of juvenile idiopathic arthritis (RF+ JIA) is a relatively rare condition (5-7% of all cases of JIA), but is associated with a poor prognosis. For these patients (pts), it is extremely important to timely prescribe adequate therapy, including biologics (B), to prevent rapid progression and early disability.ObjectivesTo analyze in retrospective study the rate of usage, efficacy and safety of B in pts with RF+ JIA.MethodsPts with RF+ JIA were identified from clinical database. Diagnosis of RF+ JIA was reviewed based on New Classification Criteria for JIA, PRINTO, 2019 [1]. Demographic and clinical data, therapy, efficacy and adverse effects observed under B were analysed.ResultsThe diagnose RF+ JIA was verified in 81 pts (11,1% boys). The median age at onset of JIA was 12.0 y.o. (7.4; 14.0). The median age at time of diagnosis – 13.0 y.o. [9.3; 15]. In total, RF+ were detected in 93.8% of pts (median 71.4 IU/ml [IQR 28.8; 218.5]), anti-cyclic citrullinated peptide antibodies-positive (ACCP+) - in 70.4% of pts (median 164.9 EU/ml [97.4; 300]). 64.2% of pts had combination RF+ and ACCP+, 29.6% - only RF+, 6.2% - only ACCP+. The therapy included NSAIDs in 97.5% of pts, steroids – in 48.1%, DMARDs in all pts (methotrexate (MTX) alone – 80.2%, 2 DMARDs – 13.6%, 3 DMARDs consecutive – 6.2%), B - in 85.2 % of pts. 2 pts received JAK-inhibitors (1 - tofacitinib, 1 – baricitinib). The median of the number of active joints at start of B was 16 [10; 23]. The median of ESR was 26 [18;43] mm/h, CRP - 14.7 [5.3;31.8] mg/l. We observed extra-articular manifestations in 29.0% of pts: 24.6% - lymphadenopathy, 4.3% - rheumatoid lung disease, 2.9% - rheumatoid nodules, 4.3% - distal polyneuropathy. Secondary Sjögren’s syndrome (SS) was diagnosed in 27.5% of pts. The experience of using B included from 1 to 4 prescriptions sequentially: 83.3% of pts received only 1 B, 20.3% - 2 B, 8.7% - 3 B, 4.3% - 4 B. B was started during the 1st year of disease in 82.7% of pts due to the rapid progression of the erosive process. As the 1st B used: abatacept (ABA) – 47.8%, etanercept (ETA) – 20.3%, adalimumab (ADA) – 10.1%, rituximab (RTM) – 7.2%, infliximab (INF) – 5.8%, tocilizumab (TCZ) – 4.3%, golimumab (GLM) – 1.4%, sarilumab – 2.9%. As the 2nd B used: 26.1% - RTM, 21.7% - ADA, 17.4% each - ETA and ABA, 13.0% - TCZ, 4.3% - GLM. As the 3d B used: 33.3 % each – TCZ and ADA, 22.2% - PTM, 11.1% - ETA. 3 pts received successively 4 B (ABA-ETA-ADA-TCZ, ABA-ETA-ADA-RTM, TCZ-ABA-ADA-RTM). Adverse events (AE) were observed in 24.6% of pts as recurrent uncomplicated upper respiratory tract infections being the most common. The reasons for substitution therapy were serious AE, subsequent loss of effect. The frequency of ACCP+ was not statistically different in groups with the effective use of only 1 B and, if necessary, replacement. In the presence of systemic manifestations, preference was given to TCZ or RTM, with secondary SS - RTM or ABA.ConclusionMost pts with RF+ JIA needs to require B in the first year of the disease. Application B has good efficacy and an acceptable safety profile. The presence of systemic manifestations/secondary SS influence on choice of B. Our data didn’t reveal the effect of ACCP+ on preferred choice or frequency of replacement B.

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