[Sjögren's syndrome with juvenile onset].

Aim to analyze demographic data, clinical features and results of laboratory and instrumental examinations in children with primary and secondary Sjögren syndrome (SS). MATERIALS AND METHODS: The study included all consequently patients, who hospitalized to the pediatric department of V.A. Nasonova Scientific and Research Institute of Rheumatology from January 2013 to December 2018, which verified the diagnosis of the SS. RESULTS: The diagnosis of SS was established in 30 patients, among whom there were only 5 (16.7%) boys, the ratio of boys and girls was 1:5. According to the results of the examination, the following diagnoses were verified: 4 - primary SS, 9 - systemic lupus erythematosus with SS, 10 - juvenile rheumatoid arthritis with SS, 3 - mixed connective tissue disease, 3 - overlap syndrome, 1 - systemic sclerosis with SS. The median age of rheumatic disease onset was 10.4 (7.0; 13.75) years. The median of disease duration at the time of SS verification - 3.0 (0.85; 4.4) years. Recurrent parotitis were observed in 8 patients. 24 pts had isolated involvement of salivary glands, 6 - combined with lacrimal glands. Sicca syndrome was occurred in 8 patients. All patients had systemic manifestations: constitutional abnormalities - 50%, polyarthritis - 83.3%, lymphadenopathy - 73.3%, cutaneous involvement - 60%, pulmonary involvement - 23.3%. Of the hematological disorders, leuko/lymphopenia was more often recorded - in 30%, polyclonal hypergammaglobulinemia - in 26.7% of patients. ANA were detected in all cases, anti-Ro antibodies - 60%, a positive rheumatoid factor - 56.7% of patients. The most common combination of immunological disorders was the presence of ANA, RF and anti-Ro antibodies (40% of patients). The treatment for each patient was justified by the main manifestations and activity of rheumatic disease: 66.7% received nonsteroidal anti - inflammatory drugs, 80% - glucocorticoids, 46.7% - methotrexate, 20% - azathioprine, 43.3% - hydroxychloroquine, 10% - mycophenolatis mofetilum, 3.4% - cyclophosphamide. 66.7% of patients received the treatment of Biologics. CONCLUSIONS: Early diagnosis of SS in children with rheumatic diseases significantly affects to the choice of treatment and prognosis. In children the SS has no clinical manifestations for a long time. Such symptoms as a hypergammaglobulinemia, positive RF without persistant arthritis, nonspecific skin lesions, recurrent parotid swelling may help to diagnosis of SS.

[Spondyloarthritis: modern terminology and definitions].

Aim to identify outdated terms and make changes to the terminology of spondyloarthritis. MATERIALS AND METHODS: At the first stage of the work, the terms divided into two categories: "outdated" definitions and terms that need to be improved or unified. Subsequently, each member of the Expert Group of Spondyloarthritis at the Association of Rheumatologists of Russia (ExSpA) presented by its own definition of the designated term or agreed with the previous term. At the next stage, the existing definitions were put together. After discussion, experts left a term that scored at least 2/3 of the votes. The special opinion of experts was recorded, whose did not coincide with the majority opinion. An open vote was conducted, when defining an "outdated" term with the unanimous decision of all group members, this term was not recommended for further clinical use. RESULTS: The work carried out allowed us to identify a number of terms that are not recommended for use in clinical practice. Number of terms are defined, which should be used when discussing the problem of spondyloarthritis. CONCLUSION: The Expert Group of Spondyloarthritis at the Association of Rheumatologists of Russia suggests using or, accordingly, not using a number of terms and their definitions in clinical practice.

[Undifferentiated spondyloarthropathies].

The article is a review of the various aspects of the undifferentiated spondyloarthropathies. Data on the prevalence denote considerable frequency of undifferentiated spondyloarthropathies in various age groups. Clinical symptoms, evolution and treatment are provided. The special attention is given to a problem of juvenile spondyloarthropathies. Changes of the nomenclature of the spondyloarthropathies are offered, allowing to improve diagnostics and treatment of these diseases.

[State-of-the-art pharmacotherapy of juvenile arthritis].

The review of state-of-the-art stage of pharmacotherapy of juvenile arthritis is represented. Main principles and features of the use of antirheumatic remedies in children are given with the accent to age limitations. Detailed characteristic used drugs and treatment schemes and also recommendations for prevention and monitoring of collateral reactions are given. Special attention is devoted to differential approach to the therapy of juvenile arthritis various variants: systemic, polyarticular and oligoarticular. Schematic algorithms of therapeutical tactics are represented.

[The current clinical and diagnostic aspects of juvenile ankylosing spondylarthritis]. / Sovremennye aspekty kliniki i diagnostiki iuvenil'nogo ankiloziruiushchego spondiloartrita.

The examination was performed of 700 children with juvenile arthritis diverse in nosology. Juvenile ankylosing spondylarthritis (JSA) was diagnosed in 60 patients. In 31 of them the diagnosis was accurate, 29 patients were suspected of having JSA by Garmisch-Partenkirchen criteria. 95% of the examinees were newly diagnosed. The study demonstrated polymorphism of clinical symptoms, variability of JSA course, articular syndrome characteristics, those of extraarticular manifestations, and the axial lesion.