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Аутовоспалительные синдромы (АВС) -группа преи-мущественно наследственных заболеваний, связанных со спонтанной неконтролируемой продукцией провоспали-тельных цитокинов [1]. Поскольку лихорадка является ос-новным, неотъемлемым симптомом АВС, их зачастую от-носят к группе периодических лихорадок [2]. Благодаря исследованиям в этой области перечень АВС постоянно обновляется, расширяются возможности подтверждающей диагностики (молекулярно-генетическое типирование), разрабатываются и внедряются новые препараты для лече-ния заболеваний этой группы. АВС являются типичными орфанными заболеваниями. Их редкость, полиморфизм клинических проявлений, зачастую отсутствие возможно-сти для подтверждающей диагностики, а также низкая ин-формированность врачей приводят к поздней верифика-ции диагноза, развитию серьезных осложнений (низко-

BackgroundJuvenile idiopathic arthritis with systemic onset (soJIA) may have monocycling, polycycling or relapsed course [1]. There were not known soJIA course predictors.ObjectivesThe aim of our study was to evaluate initial clinical or laboratory features of the patients with soJIA who had monocyclic course without biologics.MethodsIn the present study were included data about 130 soJIA patients. After selection we identified a subgroup of the soJIA patients (n=22) who successfully achieved remission without any biologic medication and were stable in the remission off-medication at least two years. The second group consisted of the patients with chronic persistent course (n=83). The remained 25 patients were excluded due to missing data or who did not meet the selection criteria. We evaluated routine clinical (fever, rash, hepatosplenomegaly, serositis, lymphadenopathy, MAS, joint involvement) and laboratorial (WBC, PLT, Hb, ferritin, ALS, AST, LDH, GGTP, ALP, albumin, Na, triglycerids, ESR, CRP, prothrombin, fibrinogen) soJIA features in the onset of the disease.ResultsPatient with monocycling course have no any differences except the ferritin level: 275 (133; 698) ng/ml vs 950 (150; 3240) ng/ml, (p=0.04), time to achievement remission 17.7 (8.2; 38.0) months vs 60.2 (36.0; 99.0) months (p=0.00002) and rare elbow involvement 4.6% vs 30.9% (p=0.01). The parameters, associated with possible monocycling course are in the table.Table. The parameters, associated with possible monocyclic course in soJIA patients.ParameterAUC (95%CI)SeSpOR (95% CI)pCRP≤3.1 mg/dl0.59 (0.48; 0.69)0.530773.6 (1.3; 10.3)0.014ESR≤ 53 mm/h0.62 (0.51; 0.71)0.90.436.8 (1.5; 31.3)0.006Ferritin ≤1340 ng/ml0.69 (0.58; 0.79)1.00.46-0.003Active joints < 80.5 (0.4; 0.6)0.820.394.6 (1.3; 16.8)0.013Onset age <7 years0.57 (0.47; 0.66)0.860.44.2 (1.5; 15.3)0.022No elbow involvement, n (%)-0.960.319.4 (1.2; 73.6)0.012ConclusionPatients with soJIA initially have quit similar clinical presentations independently the further clinical course. The prediction of possible course of soJIA is a difficult problem. Patients with soJIA with monocycling course were younger and had less impressive laboratory activity. Further investigations required.Reference:[1] Cimaz R. Systemic-onset juvenile idiopathic arthritis. Autoimmun Rev. 2016;15(9):931-4.Disclosure of InterestsNone declared

Background:JIA is the most common chronic condition in pediatric rheumatology. The cervical spine (CS) involvement is associated with severe disease activity and disability and has been recognized as a factor of a poor prognosis. Data about the CS involvement is contradictory due to silent CS involvement in some patients.Objectives:the aim of our study was to provide a clinical profile of the patients with the CS involvement.Methods:753 patients for last 10 years with JIA were analyzed. Patients were divided depending on the CS involvement, which was confirmed by clinical (pain, LOM) and radiological features (effusion in the CS joints). We evaluated active joints and routine tests, such as CRP, ESR, ANA-positivity and HLA B27Results:The CS involvement was in 101 patients (13.4%). The data are in the table. The CS involvement was more frequently associated with joints of upper body, such as TMJ (23.7% vs 2.9%, p=0.000001), shoulder (29.7% vs 2.9%, p=0.000001), elbow (34.2% vs 12.2%, p=0.000001), wrist (61.4% vs 21.8%, p=0.0000001), MCP (43.6% vs 18.4%, p=0.0000001), PIP (52.5% vs 21.3%, p=0.0000001), DIP (23.8% vs 7.1%, p=0.0000001) and hip (44.6% vs 16.6%, p=0.0000001), and ankle (60.4% vs 40.2%, p=0.0001) from lower body.ParametersCS, yes (n=101)CS, no (n=652)pFemale, n (%)69 (68.3)388 (59.5)0.092ANA-positivity, n (%)22/57 (38.6)190/403 (47.2)0.226HLA B27-positivity, n (%)12/33 (36.4)88/275 (32.0)0.613Onset age, years5.3 (2.7-10.1)6.1 (3.0- 10.4)0.241ESR, mm/h12.0 (5.0-31.0)7.0 (3.0- 18.0)0.0006CRP, mg/l3.9 (0.0- 20.0)1.1 (0.0-9.2)0.002Active joints, n (%)16.0 (9.0-28.0)5.0 (3.0-10.0)0.000000Time before remission, years2.9 (1.5-5.1)2.2 (1.1-4.6)0.046OligoarthritisPolyarthritisPsoriatic arthritisEnthesitis-related arthritisSystemic arthritis5 (5.0)48 (48.0)7 (7.0)22 (21.8)19 (18.9)199 (30.5)217 (33.3)33 (5.1)164 (25.2)39 (6.0)0.0000001Uveitis, n (%)9/76 (11.9)107/444 (24.1)0,018Oral glucocorticosteroids, n (%)37 (36.7)115/651 (17.7)0.00001Biologic, n (%)68 (67.3)283 (43.4)0.000007Remission, n (%)57 (56.4)428 (65.6)0.072Flare, n (%)10 (9.9)128/651 (19.7)0.018Conclusion:The main risk factors of CS involvement in JIA were polyarthicular and systemic arthritis, high inflammatory activity and involvement of joints of upper body. Patients with CS involvement required more often biologics.Disclosure of Interests:None declared

BackgroundMethotrexate (MTX) is a gold standard for treatment of juvenile idiopathic arthritis (JIA) patients. The main adverse events (AE) related to the MTX are intolerance, increased rate of infections, elevation of the liver enzymes, hematological abnormalities and stomatitis, which required to stop the MTX therapy [1]. In children without remission who tolerated MTX often the second-line treatment is the biologics, which may use alone or in the combination with MTX.ObjectivesThe aim of our study was to evaluate the main reasons of MTX discontinuation in JIA children who started first biologic.MethodsIn the study were included 173 non-systemic JIA patients, whom biologic therapy was prescribed firstly to previous MTX treatment. We evaluate the main reasons of MTX discontinuation after the starting of the biologic treatment, duration of MTX treatment (all and after biologic start), achievement the remission, according C. Wallace criteria and time to remission, flare of JIA. We compared two groups: i) patients with biologics with ongoing methotrexate and ii) patients with biologics alone, whom MTX was discontinued or not prescribed.ResultsDuring the trial MTX discontinued 40 (23.1%) patients: due to intolerance (n=11), other adverse events (AE) (n=10), except intolerance (mainly, frequent infection and transaminitis) and remission of JIA (n=19). Patients with onset age of JIA <4.4 years were in the risk of developing MTX intolerance (LogRank test, p=0.000001), but the younger patients usually developed other AE. Compare of the three subgroups of patients, who discontinued MTX we have not yet found any differences, except the onset age and frequency of remission, which was higher in patients who discontinued MTX due to other AE than due to intolerance.ConclusionThe main factor, associated with MTX discontinuation in JIA patients, who received first biologic was the disease onset age. Eldest age was related to higher risk of developing the MTX intolerance.References[1] van Dijkhuizen EH, Pouw JN, Scheuern A, Hügle B, Hardt S, Ganser G, Kümmerle-Deschner JB, Horneff G, Holzinger D, Bulatović Ćalasan M, Wulffraat NM. Methotrexate intolerance in oral and subcutaneous administration in patients with juvenile idiopathic arthritis: a cross-sectional, observational study. Clin Exp Rheumatol. 2016;34(1):148-54.Disclosure of InterestsNone declared