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Розглянуті сучасні підходи до проблеми уніфікації в соціальній спрямованості професійної діяльності індивіда з іншими суб’єктами узгодження стандартів професійних взаємовідносин і розвитку соціальних середовищ. З’ясовано актуальність завдання інтеграції навичок галузевого фахівця через розвиток соціальних компетентностей, встановлено специфіку «соціальності» як елемента фахових компетентностей, які забезпечують успішну діяльність галузевого професіонала. Виявлено тенденції переходу досліджень від режиму констатації і зіставлення галузевих і корпоративних стандартів «соціальних» компетентностей до теоретично обґрунтованих і емпіричним шляхом виявлених у ході багатофакторного аналізу оцінки й обліку суб’єктних складових соціальних норм у професійному середовищі. Проаналізована специфіка праці фахівців водного транспорту (морського вантажного флоту) в контексті галузевих професійних вимог і сучасних викликів. З’ясовані соціологічні параметри залежностей і відповідностей культурно-світоглядно-мотиваційних чинників професійної діяльності від динаміки і якості процесу становлення професіонала. Висновується низка процесуальних ракурсів дослідження соціальних навичок і особистих якостей фахівця водного транспорту для комплексної оцінки взаємовпливів різнорідних чинників розвитку соціально значимих функцій в професійній діяльності. Обґрунтована потреба використання соціологічних засобів поглиблення знань, деталізації соціальних аспектів життєдіяльності, оптимального вибору методології емпіричного дослідження соціальної компетентності працівників цієї професійної групи та визначення напрямів наукового і професійно-освітнього супроводу її розвитку.

Background Systemic juvenile idiopathic arthritis (SoJIA) is the most striking form of juvenile idiopathic arthritis. The aim of our study was to evaluate the clinical responses and outcomes of children with SoJIA to IL-6 blockade using two different tocilizumab (TCZ) treatment protocols designed for milder and more severe SoJIA patient groups, and evaluate the possibility of achieving biologic-free remission. Methods Thirty-seven active SoJIA children who have failed treatment with corticosteroids and other DMARDs were included in our retrospective study. TCZ doses were prescribed in two treatment approaches: every 2 weeks TCZ dosing (Q2W) and every 4 weeks TCZ dosing (Q4W). The patients were assigned to these two groups by the study physicians depending on the severity of the SoJIA disease as judged by each clinician. Results Thirty-three of the 37 children successfully completed the trial. TCZ was discontinued in 11patients during the trial. Seven children achieved inactive disease and were allowed to stop the TCZ and 4 had severe adverse events requiring drug cessation. Currently 7 patients continue to have TCZ-free remission [4/7 remission off-medication, 3/7still on methotrexate (MTX)]. This mixed group had a median treatment duration of 1002 days. The children in remission off of all medications, TCZ and MTX, had a median remission duration of 1162 days (ranged 932–1301 days). Compared to the patients assigned to the Q2W TCZ treatment group, the patients assigned to the Q4W TCZ group had a milder SoJIA course. The patients had higher levels of hemoglobin, total proteins, and serum albumins. They had lower white blood cell counts (WBC), % granulocytes, CRP, ESR, ferritins, and LDH. These children had a lower frequency of internal organ involvement, fewer relapses during TCZ treatment, and no macrophage activation syndrome episodes. Conclusions Our experience with TCZ for SoJIA supports the excellent result of other studies. What may be novel is our finding that thisIL-6 blockade with TCZ may be able to be utilized at a less frequent dosing schedule in mild SoJIA compared to severe SoJIA. We discuss other factors that may increase the probability of a patient reaching TCZ-free remission.

Background Systemic-onset juvenile idiopathic arthritis (SoJIA) is one of the most striking forms of juvenile arthritis, which can lead to severe joint disability, organ involvement and potentially could be life-threatening. Objectives The aim of our study was to evaluate clinical response to IL-6 blockade, according to different tocilizumab (TCZ) treatment, outcomes and possibility to achieve drug-free remission. Methods Our retrospective study was included 37 active SJIA children who fall corticosteroids (CS) and DMARDs and their combination and in whom TCZ was initiated in standard dosage. We used TCZ in 2 branches: every 2 weeks (Q2W) and every 4 weeks (Q4W), depended on the severity of disease, steroid-dependence, presence of MAS and organ involvement. Patients with milder SJIA course (CS≤0.2-0.3 mg/kg, no signs of MAS and no organ involvement) were considered as suitable for Q4W treatment. Only patients who had no signs of flare, exacerbation, side effects and signs of TCZ inefficacy during 4 weeks and received TCZ Q4W (n=26). Patients who successfully treated with TCZ Q4W were recognized as group of low disease activity (LDA). If any signs of inefficacy during first 4 weeks were occurred the patient was treated with TCZ Q2W (n=11). Also if patient later had any signs of insufficient efficacy of TCZ Q4W this patient was re-assessed as HDA patient and referred to appropriate group. Results From 37 children included in the trial 33 (89.1%) successfully completed it. TCZ was discontinued in 10 patients during the trial: 6 children achieved inactive disease, allowed to stop TCZ, 4 had severe adverse events. Currently 6 patients have TCZ-free remission (3/6 remission off-medication, 3/6 still on MTX) with median duration 661 days. Patients who treated Q4W had milder SJIA course: higher hemoglobin, total protein, albumin, lower WBC, granulocytes, CRP, ESR, ferritin, LDH, no new cases of macrophage activation syndrome (MAS), lower frequency of organ involvement and relapses during TCZ treatment. No patients, who required TCZ every 2 weeks, experienced TCZ-free remission by now. All cases of MAS relapses during TCZ course were only in Q2W group. Q4W patients decreased the granulocytes number intensively than Q2W. Hb>10.3 g/dl, granulocytes≤9792 cells in 1 μl, granulocytes in 1 week≤8142 cells in 1 μl, WBC in 2 weeks≤7.5*109/l, granulocytes in 2 weeks≤3975 cells in 1 μl, platelets>208*109/l, ESR≤26 mm/h, ferritin≤605 mg/ml, LDH≤571 U/l, total protein>7.2 g/dl, albumin>2.8 g/dl, absence of hepatosplenomegaly, coagulopathy, cardiorespiratory, CNS and kidney involvement, no MAS during TCZ were factors increased possibility to use TCZ Q4W and referred the patient to LDA group. Fever before and MAS after TCZ initiation, lymphadenopathy, coagulopathy, CNS dysfunction and treatment Q2W decreased possibility to achieve TCZ-off remission. Conclusions Different response to IL-6 blockade distinguishes two different subsets of SJIA. We offer the provisional criteria distinguish patients with LDA and HDA and provisional criteria probability to rich TCZ-free remission. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.2273

Objectives The purpose of our study was to detect early clinical and laboratory signs that help to discriminate macrophage activation syndrome (MAS) from active systemic juvenile idiopathic arthritis (SJIA) without MAS. Methods Our retrospective study was based on reviewing the medical charts of the children admitted to the rheumatology department with active SJIA and definite MAS ( n = 18) and without MAS ( n = 40). We evaluated the data related to SJIA and MAS at the moment of the patient׳s admission. If the patient had signs of MAS since admission or developed definite MAS later during this flare, he was referred to the main group. The children who did not have MAS during the flare episode and did not have MAS in the past medical history were in the control group. We calculated the cutoff points for MAS parameters, performed the analysis of sensitivity and specificity, identified the predictors, and provided the preliminary diagnostic rule through "the-number-of-criteria-present" approach. Results The clinical signs were relevant to MAS in SJIA: oligoarticular disease course (OR = 5.6), splenomegaly (OR = 67.6), hemorrhages (OR = 33.0), and respiratory failure (OR = 11.3). The involvement of wrist (OR = 0.2), MCP (OR = 0.1), and PIP joints (OR = 0.1) was protective against MAS development. The best cutoffs for laboratory parameters were PLT ≤ 211 × 10 9 /l, WBC ≤ 9.9 × 10 9 /l, AST > 59.7U/l, LDH > 882U/l, albumin ≤ 2.9g/dl, ferritin > 400μg/l, fibrinogen ≤ 1.8g/l, and proteinuria. The laboratory variables were more precise in the discrimination of early MAS than clinical: any 3 or more laboratory criteria provided the highest specificity (1.0) and sensitivity (1.0) and OR=2997. Conclusions We detected clinical and laboratory markers and created preliminary diagnostic (laboratory) guidelines for early discrimination of MAS in active SJIA.

Macrophage activation syndrome (MAS) - is a severe life-threatening hematological condition, complicated different rheumatic diseases. MAS is characterized by uncontrolled proliferation of T cells and macrophages, associated with decreased natural killer (NK) cells and cytotoxic T cell function due to mutations in perforin gene.