BackgroundUveitis is the most common extra-articular manifestation of juvenile idiopathic arthritis (JIA), often entirely asymptomatic but could be sight-threatening. The main predictors of uveitis in JIA are oligoarticular (OA) subtype, ANA-positivity and younger age at the JIA onset. Methotrexate (MTX) has been able to decrease the incidence of uveitis in JIA up to 2 times [1]. Treatment of JIA depends on number of active joints: NSAID and intra-articular (IAC) steroid injection are the first line-therapy for OA subtype of JIA. In Russia there is a restricted access to prolonged intra-articular steroids, such as triamcinolone hexacetamide. In the cases of relapse after short-term IAC followed by start of MTX therapy, which lead to higher proportion of patients with JIA, treated with MTX then in Europe and North America.ObjectivesTo re-evaluate the possibility of MTX to prevent the onset of uveitis in Russian JIA cohort patients with increased proportion of applying of MTX.MethodsThe clinical charts of all consecutive patients who had received a stable management for at least 2 years with or without MTX were reviewed. Patients who were given systemic medications other than MTX (except NSAID) were excluded. Patients with systemic arthritis, rheumatoid factor-positive arthritis, or enthesitis-related arthritis were also excluded. In each patient, the al least 2-year follow-up period after first visit was examined to establish whether uveitis had occurred.ResultsA total of 281 patients with a median disease duration of 3.8 year were included. One hundred and ninety one patients (68%) were treated with MTX compare to 33.9% in previous study [1]. During the at least 2-year follow-up, 64 patients (22.8%) developed uveitis, a median of 1.6 year after the disease onset. The frequency of uveitis was lower in MTX-treated than in MTX-untreated patients (11.5% vs 46.7%, respectively, OR=6.7 (95%CI:3.7-12.3), p=0.0000001). In previous study the frequency of uveitis was 10.5 in MTX-treated vs 20.2 in MTX-untreated patients [1]. Survival analysis confirmed that patients treated with MTX had a lower probability of developing uveitis (HR=4.35, p=0.000001) (Fig.). In subgroup analysis was shown that MTX more preventive in boys (HR=6.7, p=0.0007) than in girls (HR=3.6, p=0.000001); in patient with onset age more than 5 years (HR=22.2, p=0.000001) than whom disease onset less 5 years (HR=2.3, p=0.003). The data of survival analysis of MTX prevention not shown benefits depend on number of active joints: in OA (HR=4.0, p=0.000001) similar to polyarthritis (HR=3.7, p=0.02) and ANA status: ANA-positive (HR=4.4, p=0.00002) similar to ANA-negative (HR=3.6, p=0.003).ConclusionsMTX therapy may prevent the onset of uveitis in children with JIA. Further randomized controlled trial required to confirmation our results.ReferencesPapadopoulou C, Kostik M, Bohm M, Nieto-Gonzalez JC, Gonzalez-Fernandez MI, Pistorio A, Martini A, Ravelli A. Methotrexate Therapy May Prevent the Onset of Uveitis in Juvenile Idiopathic Arthritis. J Pediatr 2013;163:87...

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Evaluation of bone mineralization and bone metabolism in children with inflammatory bowel disease (IBD) in accordance with TaqI-polymorphic genotypes of vitamin D receptor (VDR) has been performed in this study. 83 children (38 girls and 54 boys) with IBD (60 with Crohn’s disease, 23 with ulcerative colitis) have been included in the study. Mean age was 13.0 years (11.0; 15.5). All patients have active phase of the disease. Bone mineral density (BMD) of lumbar spine (DEXA) have been assessed in all children. Level of serum osteocalcin (OC) as a marker of osteosynthesis, C-terminal telopeptides (CTT) as a marker of osteoresorbtion, parathyroid hormone (PTH), serum calcium, phosphorus, alkaline phosphatase and 25(OH) vitamin D was measured to evaluate bone metabolism. Molecular-genetic tests: analysis of TaqI (rs731236) polymorphism of vitamin D receptor gene (VDR) was perfomed by polymerase chain reaction with following restriction analysis. Association between molecular markers of VDR gene and bone metabolism and mineralization disturbances have been found. TT genotype of VDR gene was associated with tendency to decrease of linear growth velocity, low linear growth, decrease in osteosynthesis and increase of osteoresorbtion in this group of children. TT-genotype of TaqI-polymorphism of VDR gene can be assessed as a risk factor of bone metabolism disturbances in children with both Crohn’s disease and ulcerative colitis. C allele seems to have protective role in this group.

Семейная средиземноморская лихорадка-типичное моногенное заболевание с аутосомно-рецессивным типом наследования; обусловлено мутациями в гене MEFV, кодирующем белок пирин. Является сравнительно редкой патологией в практике педиатров и ревматологов Российской Федерации. В статье приводятся современные данные о распространенности заболевания, представлена полная на сегодняшний день клиническая картина аутовоспалительного синдрома, рассматриваются диагностические критерии и методы лечения пациентов с семейной средиземноморской лихорадкой.

Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disease with a high prevalence in some countries. The carriers of the MEFV gene causing FML are Jews, Armenians, Turks, Arabs and other nationalities of Mediterranean origin. Crimean Tatars are one of the nations that inhabit the Crimean peninsula, who do not formally belong to Mediterranean populations. Until 2016, there were no data on FMF in Crimea among the Crimean Tatar population; however, 15 new cases of FMF have been diagnosed in the Republic of Crimea in the past 2 years. The paper provides data on FML patients and information about the ethnic origin of the Crimean Tatars, explaining the possible origin of mutant alleles in the population.