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OBJECTIVE: To develop a Childhood Lupus Improvement Index (CHILI) as a tool to measure response to therapy in childhood-onset systemic lupus erythematosus (cSLE), with a focus on clinically relevant improvement (CRIc SLE ). METHODS: Pediatric nephrology and rheumatology subspecialists (n = 213) experienced in cSLE management were invited to define CRIc SLE and rate a total of 433 unique patient profiles for the presence/absence of CRIc SLE . Patient profiles included the following cSLE core response variables (CRVs): global assessment of patient well-being (patient-global), physician assessment of cSLE activity (MD-global), disease activity index score (here, we used the Systemic Lupus Erythematosus Disease Activity Index), urine protein-to-creatinine ratio, and Child Health Questionnaire physical summary score. Percentage and absolute changes in these cSLE- CRVs (baseline versus follow-up) were considered in order to develop candidate algorithms and validate their performance (sensitivity, specificity, area under the receiver operating characteristic curve [AUC] ; range 0-1). RESULTS: During an international consensus conference, unanimous agreement on a definition of CRIc SLE was achieved ; cSLE experts (n = 13) concurred (100%) that the preferred CHILI algorithm considers absolute changes in the cSLE- CRVs. After transformation to a range of 0-100, a CHILI score of ≥54 had outstanding accuracy for identifying CRIc SLE (AUC 0.93, sensitivity 81.1%, and specificity 84.2%). CHILI scores also reflect minor, moderate, and major improvement for values exceeding 15, 68, and 92, respectively (all AUC ≥0.92, sensitivity ≥93.1%, and specificity ≥73.4%). CONCLUSION: The CHILI is a new, seemingly highly accurate index for measuring CRI in cSLE over time. This index is useful to categorize the degree of response to therapy in children and adolescents with cSLE.

Supplemental material, sj-pdf-1-jso-10.1177_23971983221143244 for Gender differences in juvenile systemic sclerosis patients: Results from the international juvenile scleroderma inception cohort by Ivan Foeldvari, Jens Klotsche, Ozgur Kasapcopur, Amra Adrovic, Maria Teresa Terreri, Ana Paula Sakamoto, Valda Stanevicha, Jordi Anton, Brian M Feldman, Flavio Sztajnbok, Raju Khubchandani, Ekaterina Alexeeva, Maria Katsicas, Sujata Sawhney, Vanessa Smith, Simone Appenzeller, Tadej Avcin, Mikhail Kostik, Thomas Lehman, Edoardo Marrani, Dieneke Schonenberg-Meinema, Walter-Alberto Sifuentes-Giraldo, Natalia Vasquez-Canizares, Mahesh Janarthanan, Monika Moll, Dana Nemcova, Anjali Patwardhan, Maria Jose Santos, Cristina Battagliotti, Lillemor Berntson, Blanca Bica, Jürgen Brunner, Rolando Cimaz, Patricia Costa-Reis, Despina Eleftheriou, Liora Harel, Gerd Horneff, Sindhu R Johnson, Daniela Kaiser, Tilmann Kallinich, Dragana Lazarevic, Kirsten Minden, Susan Nielsen, Farzana Nuruzzaman, Siri Opsahl Hetlevik, Yosef Uziel, Nicola Helmus and Kathryn S Torok in Journal of Scleroderma and Related Disorders

Supplemental material, sj-pdf-1-jso-10.1177_23971983221143244 for Gender differences in juvenile systemic sclerosis patients: Results from the international juvenile scleroderma inception cohort by Ivan Foeldvari, Jens Klotsche, Ozgur Kasapcopur, Amra Adrovic, Maria Teresa Terreri, Ana Paula Sakamoto, Valda Stanevicha, Jordi Anton, Brian M Feldman, Flavio Sztajnbok, Raju Khubchandani, Ekaterina Alexeeva, Maria Katsicas, Sujata Sawhney, Vanessa Smith, Simone Appenzeller, Tadej Avcin, Mikhail Kostik, Thomas Lehman, Edoardo Marrani, Dieneke Schonenberg-Meinema, Walter-Alberto Sifuentes-Giraldo, Natalia Vasquez-Canizares, Mahesh Janarthanan, Monika Moll, Dana Nemcova, Anjali Patwardhan, Maria Jose Santos, Cristina Battagliotti, Lillemor Berntson, Blanca Bica, Jürgen Brunner, Rolando Cimaz, Patricia Costa-Reis, Despina Eleftheriou, Liora Harel, Gerd Horneff, Sindhu R Johnson, Daniela Kaiser, Tilmann Kallinich, Dragana Lazarevic, Kirsten Minden, Susan Nielsen, Farzana Nuruzzaman, Siri Opsahl Hetlevik, Yosef Uziel, Nicola Helmus and Kathryn S Torok in Journal of Scleroderma and Related Disorders

Background and aim Striving for harmonisation of specialty training and excellence of care in rheumatology, the European League Against Rheumatism (EULAR) established a task force to develop points to consider (PtCs) for the assessment of competences during rheumatology specialty training. Methods A systematic literature review on the performance of methods for the assessment of competences in rheumatology specialty training was conducted. This was followed by focus groups in five selected countries to gather information on assessment practices and priorities. Combining the collected evidence with expert opinion, the PtCs were formulated by the multidisciplinary task force, including rheumatologists, medical educationalists, and people with rheumatic and musculoskeletal diseases. The level of agreement (LoA) for each PtC was anonymously voted online. Results Four overarching principles and 10 PtCs were formulated. The overarching principles highlighted the importance of assessments being closely linked to the rheumatology training programme and protecting sufficient time and resources to ensure effective implementation. In the PtCs, two were related to overall assessment strategy (PtCs 1 and 5); three focused on formative assessment and portfolio (PtCs 2-4); three focused on the assessment of knowledge, skills or professionalism (PtCs 6-8); one focused on trainees at risk of failure (PtC 9); and one focused on training the trainers (PtC 10). The LoA (0-10) ranged from 8.75 to 9.9. Conclusion These EULAR PtCs provide European guidance on assessment methods throughout rheumatology training programmes. These can be used to benchmark current practices and to develop future strategies, thereby fostering continuous improvement in rheumatology learning and, ultimately, in patient care.

Juvenile systemic sclerosis (JSSc) is a rare disease of childhood and currently no international consensus exists with regard to its assessment and treatment. This SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) initiative, based on expert opinion informed by the best available evidence, provides recommendations for the assessment and treatment of patients with JSSc with a view to improving their outcome. Experts focused attention not only on the skin assessment but also on the early signs of internal organ involvement whose proper treatment can significantly affect the long-term outcome. A score for disease severity is proposed in order to perform a structured assessment of outcome over time but a validation in a wider patient population is recommended. Finally, a stepwise treatment approach is proposed in order to unify the standard of care throughout Europe with the aim to reduce morbidity and mortality in this disease. European Agency for Health and Consumers (EAHC) [20111202] The SHARE project was supported by a grant from the European Agency for Health and Consumers (EAHC), grant number 20111202.

Extended methodology from publication on AMRC of Prior elicitation of the efficacy and tolerability of Methotrexate and Mycophenolate Mofetil in Juvenile Localised SclerodermaSpecifically:Appendix S1 Participants in prior elicitation meetingAppendix S2 – Inclusion criteria for a clinical trial of MTX and MMF defined before elicitation exerciseAppendix S3 Detailed methodology of elicitation process

OBJECTIVE: To revise the current juvenile idiopathic arthritis (JIA) International League of Associations for Rheumatology (ILAR) classification criteria with an evidence-based approach, using clinical and routine laboratory measures available worldwide, to identify homogeneous clinical groups and to distinguish those forms of chronic arthritis typically seen only in children from the childhood counterpart of adult diseases. METHODS: The overall project consists of 4 steps. This work represents Step 1, a Delphi Web-based consensus and Step 2, an international nominal group technique (NGT) consensus conference for the new provisional Pediatric Rheumatology International Trials Organization JIA classification criteria. A future large data collection of at least 1000 new-onset JIA patients (Step 3) followed by analysis and NGT consensus (Step 4) will provide data for the evidence-based validation of the JIA classification criteria. RESULTS: In Step 1, three Delphi rounds of interactions were implemented to revise the 7 ILAR JIA categories. In Step 2, forty-seven questions with electronic voting were implemented to derive the new proposed criteria. Four disorders were proposed: (a) systemic JIA; (b) rheumatoid factor-positive JIA; (c) enthesitis/spondylitis-related JIA; and (d) early-onset antinuclear antibody-positive JIA. The other forms were gathered under the term "others." These will be analyzed during the prospective data collection using a list of descriptors to see whether the clustering of some of them could identify homogeneous entities. CONCLUSION: An international consensus was reached to identify different proposed homogeneous chronic disorders that fall under the historical term JIA. These preliminary criteria will be formally validated with a dedicated project.

Sperotto, Francesca/0000-0001-7109-0582; Kasapcopur, Ozgur/0000-0002-1125-7720; Wulffraat, Nico M/0000-0001-9548-5562; Anton, Jordi/0000-0002-8792-4219 WOS:000484452500017 PubMed ID: 30826775 In 2012, a European initiative called Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile localised scleroderma (JLS) is a rare disease within the group of paediatric rheumatic diseases (PRD) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. This study aims to provide recommendations for assessment and treatment of JLS. Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was formed, mainly from Europe, and consisted of 15 experienced paediatric rheumatologists and two young fellows. Recommendations derived from a validated systematic literature review were evaluated by an online survey and subsequently discussed at two consensus meetings using a nominal group technique. Recommendations were accepted if >= 80% agreement was reached. In total, 1 overarching principle, 10 recommendations on assessment and 6 recommendations on therapy were accepted with >= 80% agreement among experts. Topics covered include assessment of skin and extracutaneous involvement and suggested treatment pathways. The SHARE initiative aims to identify best practices for treatment of patients suffering from PRDs. Within this remit, recommendations for the assessment and treatment of JLS have been formulated by an evidence-informed consensus process to produce a standard of care for patients with JLS throughout Europe. European Agency for Health and Consumers (EAHC) [20111202] This project was supported by a grant from the European Agency for Health and Consumers (EAHC), grant number 20111202.

Objective To validate the preliminary criteria of global flare for childhood-onset SLE (cSLE). Methods Pediatricians experienced in cSLE care (n = 268) rated unique patient profiles; results of standard cSLE laboratory testing and information about the cSLE flare descriptors were presented as follows: global assessment of patient well-being, physician global assessment of disease activity (MD-global), Disease Activity Index score, protein/creatinine ratio (PCR), and erythrocyte sedimentation rate (ESR). Using rater interpretation of the course of cSLE (baseline versus followup as the gold standard), performance (sensitivity, specificity, area under the receiver operating characteristic curve [AUC]) of the preliminary flare criteria was tested. An international consensus conference was held to rank the preliminary flare criteria as per the American College of Rheumatology recommendations and delineate threshold scores for minor, moderate, and major flares. Results The accuracy of the 2 highest-ranked candidate criteria that consider absolute changes (∆) of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) or British Isles Lupus Assessment Group (BILAG) (numeric scoring: A = 12, B = 8, C = 1, and D/E = 0), MD-global, PCR, and ESR were confirmed (both AUC >0.93). For the SLEDAI-based criteria (0.5 × ∆SLEDAI + 0.45 × ∆PCR + 0.5 × ∆MD-global + 0.02 × ∆ESR) flare scores ≥6.4/3.0/0.6 constituted major/moderate/minor flares, respectively. For the BILAG-based algorithm (0.4 × ∆BILAG + 0.65 × ∆PCR + 0.5 × ∆MD-global + 0.02 × ∆ESR) flare scores ≥7.4/3.7/2.2 delineated major/moderator/minor flares, respectively. These threshold values (SLEDAI, BILAG) were all >82% sensitive and specific for capturing flare severity. Conclusion Provisional criteria for global flares in cSLE are available to identify patients who experienced a flare. These criteria also allow for discrimination of the severity of cSLE exacerbations.

Systemic lupus erythematosus (SLE) is the paradigm of systemic autoimmune diseases characterised by a wide spectrum of clinical manifestations with an unpredictable relapsing-remitting course. The aim of the present work was to identify current available clinical practice guidelines (CPGs) for SLE, to provide their review and to identify physicians' and patients' unmet needs. Twenty-three original guidelines published between 2004 and 2017 were identified. Many aspects of disease management are covered, including global disease management, lupus nephritis and neuropsychiatric involvement, management of pregnancies, vaccinations and comorbidities monitoring. Unmet needs relate with disease management of some clinical manifestations and adherence to treatment. Many patient's unmet needs have been identified starting with faster diagnosis, need for more therapeutic options, guidelines on lifestyle issues, attention to quality of life and adequate education.

Objectives. To provide an overview of the paediatric rheumatology (PR) services in Europe, describe current delivery of care and training, set standards for care, identify unmet needs and inform future specialist service provision. Methods. An online survey was developed and presented to national coordinating centres of the Paediatric Rheumatology International Trials Organisation (PRINTO) (country survey) and to individual PR centres (centre and disease surveys) as a part of the European Union (EU) Single Hub and Access point for paediatric Rheumatology in Europe project. The survey contained components covering the organization of PR care, composition of teams, education, health care and research facilities and assessment of needs. Results. Response rates were 29/35 (83%) for country surveys and 164/288 (57%) for centre surveys. Across the EU, approximately one paediatric rheumatologist is available per million population. In all EU member states there is good access to specialist care and medications, although biologic drug availability is worse in Eastern European countries. PR education is widely available for physicians but is insufficient for allied health professionals. The ability to participate in clinical trials is generally high. Important gaps were identified, including lack of standardized clinical guidelines/recommendations and insufficient adolescent transition management planning. Conclusion. This study provides a comprehensive description of current specialist PR service provision across Europe and did not reveal any major differences between EU member states. Rarity, chronicity and complexity of diseases are major challenges to PR care. Future work should facilitate the development, dissemination and implementation of standards of care, treatment and service recommendations to further improve patient-centred health care across Europe.

Objective To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). Methods A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA-associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference. Results Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4%). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82% consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (κ = 0.76). Conclusion We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies.

BACKGROUND: Most data for treatment of dermatomyositis and juvenile dermatomyositis are from anecdotal, non-randomised case series. We aimed to compare, in a randomised trial, the efficacy and safety of prednisone alone with that of prednisone plus either methotrexate or ciclosporin in children with new-onset juvenile dermatomyositis. METHODS: We did a randomised trial at 54 centres in 22 countries. We enrolled patients aged 18 years or younger with new-onset juvenile dermatomyositis who had received no previous treatment and did not have cutaneous or gastrointestinal ulceration. We randomly allocated 139 patients via a computer-based system to prednisone alone or in combination with either ciclosporin or methotrexate. We did not mask patients or investigators to treatment assignments. Our primary outcomes were the proportion of patients achieving a juvenile dermatomyositis PRINTO 20 level of improvement (20% improvement in three of six core set variables at 6 months), time to clinical remission, and time to treatment failure. We compared the three treatment groups with the Kruskal-Wallis test and Friedman's test, and we analysed survival with Kaplan-Meier curves and the log-rank test. Analysis was by intention to treat. Here, we present results after at least 2 years of treatment (induction and maintenance phases). This trial is registered with ClinicalTrials.gov, number NCT00323960. FINDINGS: Between May 31, 2006, and Nov 12, 2010, 47 patients were randomly assigned prednisone alone, 46 were allocated prednisone plus ciclosporin, and 46 were randomised prednisone plus methotrexate. Median duration of follow-up was 35·5 months. At month 6, 24 (51%) of 47 patients assigned prednisone, 32 (70%) of 46 allocated prednisone plus ciclosporin, and 33 (72%) of 46 administered prednisone plus methotrexate achieved a juvenile dermatomyositis PRINTO 20 improvement (p=0·0228). Median time to clinical remission was 41·9 months in patients assigned prednisone plus methotrexate but was not observable in the other two treatment groups (2·45 fold [95% CI 1·2-5·0] increase with prednisone plus methotrexate; p=0·012). Median time to treatment failure was 16·7 months in patients allocated prednisone, 53·3 months in those assigned prednisone plus ciclosporin, but was not observable in patients randomised to prednisone plus methotrexate (1·95 fold [95% CI 1·20-3·15] increase with prednisone; p=0·009). Median time to prednisone discontinuation was 35·8 months with prednisone alone compared with 29·4-29·7 months in the combination groups (p=0·002). A significantly greater proportion of patients assigned prednisone plus ciclosporin had adverse events, affecting the skin and subcutaneous tissues, gastrointestinal system, and general disorders. Infections and infestations were significantly increased in patients assigned prednisone plus ciclosporin and prednisone plus methotrexate. No patients died during the study. INTERPRETATION: Combined treatment with prednisone and either ciclosporin or methotrexate was more effective than prednisone alone. The safety profile and steroid-sparing effect favoured the combination of prednisone plus methotrexate. FUNDING: Italian Agency of Drug Evaluation, Istituto Giannina Gaslini (Genoa, Italy), Myositis Association (USA).

Made available in DSpace on 2018-11-28T06:57:31Z (GMT). No. of bitstreams: 0 Previous issue date: 2017-05-01 Intramural NIH HHS To develop response criteria for juvenile dermatomyositis (DM). We analysed the performance of 312 definitions that used core set measures from either the International Myositis Assessment and Clinical Studies Group (IMACS) or the Paediatric Rheumatology International Trials Organisation (PRINTO) and were derived from natural history data and a conjoint analysis survey. They were further validated using data from the PRINTO trial of prednisone alone compared to prednisone with methotrexate or cyclosporine and the Rituximab in Myositis (RIM) trial. At a consensus conference, experts considered 14 top candidate criteria based on their performance characteristics and clinical face validity, using nominal group technique. Consensus was reached for a conjoint analysis-based continuous model with a total improvement score of 0-100, using absolute per cent change in core set measures of minimal (>= 30), moderate (>= 45), and major (>= 70) improvement. The same criteria were chosen for adult DM/polymyositis, with differing thresholds for improvement. The sensitivity and specificity were 89% and 91-98% for minimal improvement, 92-94% and 94-99% for moderate improvement, and 91-98% and 85-86% for major improvement, respectively, in juvenile DM patient cohorts using the IMACS and PRINTO core set measures. These criteria were validated in the PRINTO trial for differentiating between treatment arms for minimal and moderate improvement (p= 0.009-0.057) and in the RIM trial for significantly differentiating the physician's rating for improvement (p< 0.006). The response criteria for juvenile DM consisted of a conjoint analysis-based model using a continuous improvement score based on absolute per cent change in core set measures, with thresholds for minimal, moderate, and major improvement. NIH, NIEHS, Bethesda, MD USA Univ Pittsburgh, Pittsburgh, PA 15260 USA Ist Giannina Gaslini, Genoa, Italy Social & Sci Syst Inc, Durham, NC USA Hosp Sick Children, Toronto, ON, Canada IWK Hlth Ctr, Halifax, NS, Canada Univ Florence, Florence, Italy Univ Buenos Aires, Hosp Ninos Pedro Elizalde, Buenos Aires, DF, Argentina Univ Fed Rio de Janeiro, Rio De Janeiro, Brazil Univ Kansas, Med Ctr, Kansas City, KS 66103 USA Great Ormond St Hosp Sick Children, Children NHS Trust, London, England Univ Texas Dallas, Southwester Med Ctr, Dallas, TX 75083 USA Univ Genoa, Ist Giannina Gaslini, Pediatria Reumatol 2, Genoa, Italy Duke Univ, Durham, NC 27706 USA Emory Univ, Sch Med, Atlanta, GA 30322 USA Univ Med Ctr Utrecht, Wilhelmina Childrens Hosp, Utrecht, Netherlands Hannover Med Sch, Hannover, Germany Univ Estadual Paulista, Sao Paulo, Brazil Semmelweis Univ, Budapest, Hungary Royal Hosp Sick Children, Glasgow, Lanark, Scotland Royal Hosp Sick Children, Edinburgh, Midlothian, Scotland Karolinska Univ Hosp, Stockholm, Sweden Hosp Pediatria Garrahan, Buenos Aires, DF, Argentina Ist Giannina Gaslini, PRINTO, Pediatria Reumatol 2, Genoa, Italy Charles Univ Prague, Prague, Czech Republic Univ Estadual Paulista, Sao Paulo, Brazil Intramural NIH HHS: ZIA ES101081-13 Intramural NIH HHS: ZIA ES101081-14 Intramural NIH HHS: Z99 ES999999 Intramural NIH HHS: ZIA ES101081-15 Intramural NIH HHS: ZIA ES101081-12

Istituto G. Gaslini Paediatric Rheumatology International Trials Organisation (PRINTO), Genoa, Italy Charles University Grant Agency European League Against Rheumatism (EULAR) Scientific Training Bursaries Background: Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), belongs to the group of ANCA-associated necrotizing vasculitides. This study describes the clinical picture of the disease in a large cohort of GPA paediatric patients.Children with age at diagnosis 50 patients) derived from the literature.Findings: the 56 children with GPA/WG were predominantly females (68%) and Caucasians (82%) with a median age at disease onset of 11.7 years, and a median delay in diagnosis of 4.2 months. the most frequent organ systems involved before/at the time of diagnosis were ears, nose, throat (91%), constitutional (malaise, fever, weight loss) (89%), respiratory (79%), mucosa and skin (64%), musculoskeletal (59%), and eye (35%), 67% were ANCA-PR3 positive, while haematuria/proteinuria was present in > 50% of the children. in adult series, the frequency of female involvement ranged from 29% to 50% with lower frequencies of constitutional (fever, weight loss), ears, nose, throat (oral/nasal ulceration, otitis/aural discharge), respiratory (tracheal/endobronchial stenosis/obstruction), laboratory involvement and higher frequency of conductive hearing loss than in this paediatric series.Conclusions: Paediatric patients compared to adults with GPA/WG have similar pattern of clinical manifestations but different frequencies of organ involvement. PRINTO, Ist Giannina Gaslini Pediat Reumatol 2, Genoa, Italy Charles Univ Prague, Fac Med 1, Prague, Czech Republic Gen Univ Hosp Prague, Prague, Czech Republic Hacettepe Univ, Childrens Hosp, Dept Pediat Rheumatol & Nephrol, Ankara, Turkey Ist Giannina Gaslini, Serv Epidemiol & Biostat, I-16148 Genoa, Italy NHS Fdn Trust, Great Ormond St Hosp, Dept Rheumatol, London, England Ist Giannina Gaslini, I-16148 Genoa, Italy Charite, Dept Pediat, Div Pneumol & Immunol, D-13353 Berlin, Germany Ann & Robert H Lurie Childrens Hosp Chicago, Chicago, IL USA Univ Paris 05, Unit Immunol Hematol & Rhumatol Pediat, Ctr Reference Natl Arthrit Juveniles, Hop Necker Enfants Malad,Inst IMAGINE, Paris, France Univ Gothenburg, Dept Pediat, Queen Silvia Childrens Hosp, Gothenburg, Sweden Aarhus Univ Hosp, Skejby Sygehus, Dept Pediat, Pediat Rheumatol Clin, DK-8000 Aarhus, Denmark Universidade Federal de São Paulo, São Paulo, Brazil Rigshosp, Juliane Marie Centret, Pediat Klin 2, DK-2100 Copenhagen, Denmark Emma Children Hosp AMC, Dept Pediat, Amsterdam, Netherlands Univ Childrens Hosp, Univ Med Ctr Ljubljana, Dept Allergol Rheumatol & Clin Immunol, Ljubljana, Slovenia Hosp Dr Felipe Glasman, Rheumatolgy Sect, Buenos Aires, DF, Argentina Klinikum Eilbek Hs 6, Hamburger Zentrum Kinder & Jugendrheumatol, Hamburg, Germany Univ Childrens Hosp, Dept Pediat Rheumatol & Immunol, Munster, Germany Univ Hosp Ctr, Dept Pediat, Tirana, Albania Univ Paris 11, APHP, CHU Le Kremlin Bicetre, CEREMAI Ctr Reference Natl Malad Autoinflammat Rh, Paris, France Asklepios Klin Altona, Hamburg, Germany Kinderklin Garmisch Partenkirchen gGmbH, Deutsch Zentrum Kinder & Jugendrheumatol, Garmisch Partenkirchen, Germany Wilhelmina Childrens Hosp, Dept Pediat Immunol & Rheumatol, Utrecht, Netherlands Benghazi Children Hosp Benghazi, MUB Rheumatol Clin, Benghazi, Libya Univ Genoa, Dipartimento Pediat, Genoa, Italy Pediatrics, Universidade Federal de São Paulo, São Paulo, Brazil Charles University Grant Agency: GAUK 52608/2008 Web of Science

Objective. To describe the clinical, laboratory, and histopathologic features, current treatment, and outcome of patients with macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (JIA). Methods. In this multinational, multicenter study, pediatric rheumatologists and hemato-oncologists entered patient data collected retrospectively into a web-based database. Results. A total of 362 patients, 22% of whom had MAS at the onset of systemic JIA, were included in the study by 95 investigators from 33 countries. The most frequent clinical manifestations were fever (96%), hepatomegaly (70%), and splenomegaly (58%). Central nervous system dysfunction and hemorrhages were recorded in 35% and 20% of the patients, respectively. Platelet count and liver transaminase, ferritin, lactate dehydrogenase, triglyceride, and D-dimer levels were the sole laboratory biomarkers showing a percentage change of >50% between the pre-MAS visit and MAS onset. Evidence of macrophage hemophagocytosis was found in 60% of the patients who underwent bone marrow aspiration. MAS occurred most frequently in the setting of active underlying disease, in the absence of a specific trigger. Nearly all patients were given corticosteroids, and 61% received cyclosporine. Biologic medications and etoposide were given to 15% and 12% of the patients, respectively. Approximately one-third of the patients required admission to the intensive care unit (ICU), and the mortality rate was 8%. Conclusion. This study provides information on the clinical spectrum and current management of systemic JIA-associated MAS through the analysis of a very large patient sample. MAS remains a serious condition, as a sizeable proportion of patients required admission to the ICU or died.

Objective was to describe the clinical, laboratory, and histopathologic features, current treatment, and outcome of patients with macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (JIA). In this multinational, multicenter study, pediatric rheumatologists and hemato-oncologists entered patient data collected retrospectively into a web-based database. A total of 362 patients, 22% of whom had MAS at the onset of systemic JIA, were included in the study by 95 investigators from 33 countries. The most frequent clinical manifestations were fever (96%), hepatomegaly (70%), and splenomegaly (58%). Central nervous system dysfunction and hemorrhages were recorded in 35% and 20% of the patients, respectively. Platelet count and liver transaminase, ferritin, lactate dehydrogenase, triglyceride, and d-dimer levels were the sole laboratory biomarkers showing a percentage change of >50% between the pre-MAS visit and MAS onset. Evidence of macrophage hemophagocytosis was found in 60% of the patients who underwent bone marrow aspiration. MAS occurred most frequently in the setting of active underlying disease, in the absence of a specific trigger. Nearly all patients were given corticosteroids, and 61% received cyclosporine. Biologic medications and etoposide were given to 15% and 12% of the patients, respectively. Approximately one-third of the patients required admission to the intensive care unit (ICU), and the mortality rate was 8%. This study provides information on the clinical spectrum and current management of systemic JIA–associated MAS through the analysis of a very large patient sample. MAS remains a serious condition, as a sizeable proportion of patients required admission to the ICU or died.

Made available in DSpace on 2015-03-18T15:55:50Z (GMT). No. of bitstreams: 0 Previous issue date: 2014-11-25Bitstream added on 2015-03-18T16:28:34Z : No. of bitstreams: 1 WOS000346520700001.pdf: 205186 bytes, checksum: c29e39dcb1d87ab0b78ebf2affc7a14b (MD5) Background: Rheumatic diseases in children are associated with significant morbidity and poor health-related quality of life (HRQOL). There is no health-related quality of life (HRQOL) scale available specifically for children with less common rheumatic diseases. These diseases share several features with systemic lupus erythematosus (SLE) such as their chronic episodic nature, multi-systemic involvement, and the need for immunosuppressive medications. HRQOL scale developed for pediatric SLE will likely be applicable to children with systemic inflammatory diseases.Findings: We adapted Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY (c)) to Simple Measure of Impact of Illness in Youngsters (SMILY (c)-Illness) and had it reviewed by pediatric rheumatologists for its appropriateness and cultural suitability. We tested SMILY (c)-Illness in patients with inflammatory rheumatic diseases and then translated it into 28 languages. Nineteen children (79% female, n= 15) and 17 parents participated. The mean age was 12 +/- 4 years, with median disease duration of 21 months (1-172 months). We translated SMILY (c)-Illness into the following 28 languages: Danish, Dutch, French (France), English (UK), German (Germany), German (Austria), German (Switzerland), Hebrew, Italian, Portuguese (Brazil), Slovene, Spanish (USA and Puerto Rico), Spanish (Spain), Spanish (Argentina), Spanish (Mexico), Spanish (Venezuela), Turkish, Afrikaans, Arabic (Saudi Arabia), Arabic (Egypt), Czech, Greek, Hindi, Hungarian, Japanese, Romanian, Serbian and Xhosa.Conclusion: SMILY (c)-Illness is a brief, easy to administer and score HRQOL scale for children with systemic rheumatic diseases. It is suitable for use across different age groups and literacy levels. SMILY (c)-Illness with its available translations may be used as useful adjuncts to clinical practice and research. Rutgers State Univ, Robert Wood Johnson Med Sch, New Brunswick, NJ 08903 USA Rutgers State Univ, Child Hlth Inst New Jersey, New Brunswick, NJ 08901 USA Hosp Special Surg, New York, NY 10021 USA Univ Michigan, Ann Arbor, MI 48109 USA Red Cross War Mem Childrens Hosp, Cape Town, South Africa Ain Shams Univ, Pediat Allergy Immunol & Rheumatol Unit, Cairo, Egypt Ain Shams Univ, Pediat Rheumatol Pediat Allergy Immunol & Rheum, Cairo, Egypt King Faisal Specialist Hosp & Res Ctr, Riyadh 11211, Saudi Arabia Charles Univ Prague, Prague, Czech Republic Gen Univ Hosp, Prague, Czech Republic Univ Hosp Motol, Dept Pediat, Prague, Czech Republic Aarhus Univ, Hosp Skejby, Aarhus, Denmark Rigshosp, Juliane Marie Ctr, DK-2100 Copenhagen, Denmark Univ Med Ctr, Dept Pediat Immunol, Utrecht, Netherlands Wilhelmina Childrens Hosp, Utrecht, Netherlands Great Ormond St Hosp Sick Children, Children NHS Fdn Trust, Renal Unit, London, England Lyon Univ, Hosp Civils Lyon, Rheumatol & Dermatol Dept, Lyon, France Med Univ Innsbruck, A-6020 Innsbruck, Austria Prim Univ Doz, Bregenz, Austria Hamburg Ctr Pediat & Adolescence Rheumatol, Hamburg, Germany Asklepios Clin Sankt, Augustin, Germany Univ Zurich, Childrens Hosp, Zurich, Switzerland Aristotle Univ Thessaloniki, Pediat Immunol & Rheumatol Referral Ctr, GR-54006 Thessaloniki, Greece Israel Meir Hosp, Kefar Sava, Israel Sanjay Gandhi Postgrad Inst Med Sci, Lucknow, Uttar Pradesh, India Semmelweis Univ, H-1085 Budapest, Hungary Anna Meyer Hosp, Florence, Italy Univ Siena, Res Ctr System Autoimmune & Autoinflammatory Dis, I-53100 Siena, Italy Univ Florence, Florence, Italy Osped Pediat Bambino Gesu, IRCCS, Pediat Rheumatol Unit, Rome, Italy Univ Genoa Pediat II Reumatol, Ist G Gaslini EULAR, Ctr Excellence Rheumatol, Genoa, Italy Univ Cattolica Sacro Cuore, Inst Pediat, Rome, Italy Univ Padua, Dept Pediat, Pediat Rheumatol Unit, Padua, Italy Yokohama City Univ, Sch Med, Yokohama, Kanagawa 232, Japan Univ Estadual Paulista, UNESP, Botucatu, SP, Brazil Univ Fed Sao Paulo, Dept Pediat, Sao Paulo, Brazil Univ Estadual Campinas, Dept Med, Campinas, SP, Brazil Univ Fed Rio de Janeiro, Dept Pediat, Rio De Janeiro, Brazil Univ Estado do, Adolescent Hlth Care Unit, Div Pediat Rheumatol, Rio De Janeiro, Brazil Univ Sao Paulo, Fac Med, Childrens Inst, Dept Pediat,Pediat Rheumatol Unit, Sao Paulo, Brazil Childrens Inst, Pediat Rheumatol Unit, Sao Paulo, Brazil Clin Pediat I, Cluj Napoca, Romania Inst Rheumatol, Belgrade, Serbia Univ Childrens Hosp, Univ Med Ctr Ljubljana, Ljubljana, Slovenia Head Rheumatol Hosp Pedro Elizalde, Buenos Aires, DF, Argentina Hosp Gen Mexico City, Mexico City, DF, Mexico Hosp Infantil Mexico Fed Gomez, Mexico City, DF, Mexico Hosp San Juan Dios, Barcelona, Spain Hosp Univ Valle Hebron, Barcelona, Spain Mt Sinai Med Ctr, New York, NY 10029 USA Mt Sinai Med Ctr, Miami Beach, FL 33140 USA Complejo Hosp Univ Ruiz & Paez, Bolivar, Venezuela Hacettepe Univ, Dept Pediat, Ankara, Turkey Istanbul Univ, Cerrahpasa Med Sch, Istanbul, Turkey FMF Arthrit Vasculitis & Orphan Dis Res Ctr, Inst Hlth Sci, Ankara, Turkey Univ Calgary, Dept Pediat, Alberta Childrens Hosp, Res Inst, Calgary, AB T2N 1N4, Canada Univ Estadual Paulista, UNESP, Botucatu, SP, Brazil

Background: To derive a list of opportunistic infections (OI) through the analysis of the juvenile idiopathic arthritis (JIA) patients in the Pharmachild registry by an independent Safety Adjudication Committee (SAC). Methods: The SAC (3 pediatric rheumatologists and 2 pediatric infectious disease specialists) elaborated and approved by consensus a provisional list of OI for use in JIA. Through a 5 step-procedure, all the severe and serious infections, classified as per MedDRA dictionary and retrieved in the Pharmachild registry, were evaluated by the SAC by answering six questions and adjudicated with the agreement of 3/5 specialists. A final evidence-based list of OI resulted by matching the adjudicated infections with the provisional list of OI. Results: A total of 772 infectious events in 572 eligible patients, of which 335 serious/severe/very severe non-OI and 437 OI (any intensity/severity), according to the provisional list, were retrieved. Six hundred eighty-two of 772 (88.3%) were adjudicated as infections, of them 603/682 (88.4%) as common and 119/682 (17.4%) as OI by the SAC. Matching these 119 opportunistic events with the provisional list, 106 were confirmed by the SAC as OI, and among them infections by herpes viruses were the most frequent (68%), followed by tuberculosis (27.4%). The remaining events were divided in the groups of non-OI and possible/patient and/or pathogen-related OI. Conclusions: We found a significant number of OI in JIA patients on immunosuppressive therapy. The proposed list of OI, created by consensus and validated in the Pharmachild cohort, could facilitate comparison among future pharmacovigilance studies. © The Author(s). 2020 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Made available in DSpace on 2019-10-05T16:54:20Z (GMT). No. of bitstreams: 0 Previous issue date: 2019-04-01 IRCCS Istituto Giannina Gaslini Background To our knowledge, the characteristics and burden of childhood arthritis have never been studied on a worldwide basis. We aimed to investigate, with a cross-sectional study, the prevalence of disease categories, treatment methods, and disease status in patients from across different geographical areas and from countries with diverse wealth status. Methods In this multinational, cross-sectional, observational cohort study, we asked international paediatric rheumatologists from specialised centres to enrol children with a diagnosis of juvenile idiopathic arthritis, according to International League of Associations for Rheumatology criteria, who were seen consecutively for a period of 6 months. Each patient underwent retrospective and cross-sectional assessments, including measures of disease activity and damage and questionnaires on the wellbeing and quality of life of the children. We qualitatively compared the collected data across eight geographical areas, and we explored an association between disease activity and damage and a country's gross domestic product (GDP) with a multiple logistic regression analysis. Findings Between April 4, 2011, and Nov 21, 2016, 9081 patients were enrolled at 130 centres in 49 countries, grouped into eight geographical areas. Systemic arthritis (125 [33.0%] of 379 patients) and enthesitis-related arthritis (113 [29.8%] of 379) were more common in southeast Asia, whereas oligoarthritis was more prevalent in southern Europe (1360 [56.7%] of 2400) and rheumatoid factor-negative polyarthritis was more frequent in North America (165 [31.5%] of 523) than in the other areas. Prevalence of uveitis was highest in northern Europe (161 [19.1%] of 845 patients) and southern Europe (450 [18.8%] of 2400) and lowest in Latin America (54 [6.4%] of 849), Africa and Middle East (71 [5.9%] of 1209), and southeast Asia (19 [5.0%] of 379). Median age at disease onset was lower in southern Europe (3.5 years, IQR 1.9-7.3) than in other regions. Biological, disease-modifying antirheumatic drugs were prescribed more frequently in northern Europe and North America than in other geographical settings. Patients living in countries with lower GDP had greater disease activity and damage than those living in wealthier countries. Damage was associated with referral delay. Interpretation Our study documents a variability in prevalence of disease phenotypes and disparities in therapeutic choices and outcomes across geographical areas and wealth status of countries. The greater disease burden in lowerresource settings highlights the need for public health efforts aimed at improving equity in access to effective treatments and care for juvenile idiopathic arthritis. Copyright (C) 2019 Elsevier Ltd. All rights reserved. Univ Genoa, Ist Giannina Gaslini, IRCCS, Clin Paediat & Rheumatol, Genoa, Italy Univ Genoa, Ist Giannina Gaslini, IRCCS, Epidemiol & Biostat Serv, Genoa, Italy Univ Genoa, Ist Giannina Gaslini, IRCCS, Sci Directory, Genoa, Italy Univ Genoa, PRINTO, IRCCS, Ist Giannina Gaslini, Genoa, Italy Univ Genoa, Dept Neurosci Rehabil Ophthalmol Genet & Maternal, Genoa, Italy Wilhelmina Childrens Hosp, Dept Pediat Immunol & Rheumatol, Utrecht, Netherlands Sanjay Gandhi Postgrad Inst Med Sci, Lucknow, Uttar Pradesh, India Univ Hosp 12 Octubre, Dept Pediat Rheumatol, Madrid, Spain Alfaisal Univ, Dept Pediat Rheumatol, King Faisal Specialist Hosp, Riyadh, Saudi Arabia Alfaisal Univ, Res Ctr, Riyadh, Saudi Arabia Western Univ Childrens Hosp, Hlth Sci Ctr, London, ON, Canada Oslo Univ Hosp, Dept Rheumatol, Oslo, Norway Oslo Univ Hosp, Med Fac, Oslo, Norway Univ Oslo, Oslo, Norway Oslo Univ Hosp, Norwegian Natl Advisory Unit Rheumat Dis Children, Oslo, Norway Univ Hosp Munster, Dept Pediat Rheumatol & Immunol, Munster, Germany Hosp Sor Maria Ludovica, Rheumatol Serv, La Plata, Buenos Aires, Argentina Bucharest Emergency Hosp, Cluj Napoca, Romania Childrens Emergency Hosp, Cluj Napoca, Romania Cincinnati Childrens Hosp Med Ctr, Div Rheumatol, Cincinnati, OH 45229 USA Alberta Childrens Prov Gen Hosp, Div Pediat Rheumatol, Dept Pediat, Calgary, AB, Canada Sofiamed, Pediat Dept, Sofia, Bulgaria Rigshosp, Juliane Marie Ctr, Paediat Rheumatol Unit, Copenhagen, Denmark Natl Inst Rheumatism & Physiotherapy, Clin Immunol Adult & Paediat Rheumatol Dept, Budapest, Hungary Univ Latvia, Pediat Dept, Riga, Latvia Univ Latvia, Univ Childrens Hosp, Riga, Latvia Univ Estadual Paulista, Botucatu Fac Med, Botucatu, SP, Brazil Shariati Hosp, Rheumatol Res Ctr, Dept Pediat & Rheumatol, Tehran, Iran Inst Rheumatol Belgrade, Div Pediat Rheumatol, Belgrade, Serbia Thessaloniki Univ, Dept Pediat 1, Hippokrat Gen Hosp, Sch Med, Thessaloniki, Greece Univ Estadual Paulista, Botucatu Fac Med, Botucatu, SP, Brazil

Background Absent T-cell immunity resulting in life-threatening infections provides a clear rationale for hematopoetic stem cell transplantation (HSCT) in patients with severe combined immunodeficiency (SCID). Combined immunodeficiencies (CIDs) and "atypical" SCID show reduced, not absent T-cell immunity. If associated with infections or autoimmunity, they represent profound combined immunodeficiency (P-CID), for which outcome data are insufficient for unambiguous early transplant decisions. Objectives We sought to compare natural histories of severity-matched patients with/without subsequent transplantation and to determine whether immunologic and/or clinical parameters may be predictive for outcome. Methods In this prospective and retrospective observational study, we recruited nontransplanted patients with P-CID aged 1 to 16 years to compare natural histories of severity-matched patients with/without subsequent transplantation and to determine whether immunologic and/or clinical parameters may be predictive for outcome. Results A total of 51 patients were recruited (median age, 9.6 years). Thirteen of 51 had a genetic diagnosis of "atypical" SCID and 14 of 51 of CID. About half of the patients had less than 10% naive T cells, reduced/absent T-cell proliferation, and at least 1 significant clinical event/year, demonstrating their profound immunodeficiency. Nineteen patients (37%) underwent transplantation within 1 year of enrolment, and 5 of 51 patients died. Analysis of the HSCT decisions revealed the anticipated heterogeneity, favoring an ongoing prospective matched-pair analysis of patients with similar disease severity with or without transplantation. Importantly, so far neither the genetic diagnosis nor basic measurements of T-cell immunity were good predictors of disease evolution. Conclusions The P-CID study for the first time characterizes a group of patients with nontypical SCID T-cell deficiencies from a therapeutic perspective. Because genetic and basic T-cell parameters provide limited guidance, prospective data from this study will be a helpful resource for guiding the difficult HSCT decisions in patients with P-CID.

Apie 70% vaikų, sergančių lėtiniais artritais, suaugusiųjų amžių pasiekia su tam tikru negalios lygiu ir kasdienės veiklos apribojimu. Todėl ypač aktualu nagrinėti vaikams lėtinių artritų priežastis bei prognostinius veiksnius ankstyvose sąnarių ligos stadijose. Mūsų darbo tikslas buvo nustatyti ar ūminėje ligos fazėje nustatomų laboratorinių, imunologinių ir interleukinų (MRP8/14 (kalprotektinas), IL-6, IL-33) rodiklių pokyčiai, funkcinės būklės vertinimas bei ligos aktyvumas gali prognozuoti vaikams lėtinę sąnarių ligą. Atliktas tyrimas parodė, kad artritu susirgusiems vaikams MRP8/14 (kalprotektino) ir Il-6 koncentracijos buvo reikšmingai didesnės nei kontrolinės grupės vaikams tiek serume, tiek sinoviniame skystyje (p 5785 ng/ml) ir IL-6 (> 5,5 pg/ml) koncentracijos ūminėje artrito stadijoje buvo nustatytos tiems pacientams, kuriems sąnarių liga įgavo lėtinę eigą. Vertinome tai, kaip reikšmingus lėtinės sąnarių ligos kriterijus. Šiame darbe sergančių artritų vaikų funkcinė būklė ir vaiko gyvenimo kokybė pirmą kartą Lietuvoje buvo įvertinta naujai įdiegiamu ne tik Lietuvoje, bet ir pasaulyje, įvairiapusiu jaunatvinių artritų vertinimo klausimynu JAMAR, bei duomenys palyginti su seniau Lietuvoje naudojamu vaiko sveikatos būklės vertinimo klausimynu... [toliau žr. visą tekstą] The earlier studies proved that nearly 70% of children with chronic arthritis reach the adulthood with a certain level of disability and restriction of daily activities. Therefore, it is very important to analyse the causes of arthritis and prognostic factors in the early stages of disease. The aim of our study was to determine whether the changes of laboratory, immunological and interleukins (MRP8/14 (calprotectin), IL-6, IL-33) indexes, assessment of functional condition, and disease activity may predict a chronic arthritis in children. The study showed that MRP8/14 (calprotectin) and Il-6 concentrations in the serum and synovial fluid of children with arthritis were significantly higher than in the control group of healthychildren (p 5785ng/ml) and IL-6 (> 5.5 pg/ml) at the acute stage of arthritis were determined for those patients, whose were developed chronic arthritis. It was evaluated as significant criterion of the chronic joint disease. For the first time in Lithuania, the functional ability and the quality of life in children with arthritis were assessed using a newly introduced multidimensional assessment questionnaire for juvenile arthritis (JAMAR), and data was compared... [to full text]

Apie 70% vaikų, sergančių lėtiniais artritais, suaugusiųjų amžių pasiekia su tam tikru negalios lygiu ir kasdienės veiklos apribojimu. Todėl ypač aktualu nagrinėti vaikams lėtinių artritų priežastis bei prognostinius veiksnius ankstyvose sąnarių ligos stadijose. Mūsų darbo tikslas buvo nustatyti ar ūminėje ligos fazėje nustatomų laboratorinių, imunologinių ir interleukinų (MRP8/14 (kalprotektinas), IL-6, IL-33) rodiklių pokyčiai, funkcinės būklės vertinimas bei ligos aktyvumas gali prognozuoti vaikams lėtinę sąnarių ligą. Atliktas tyrimas parodė, kad artritu susirgusiems vaikams MRP8/14 (kalprotektino) ir Il-6 koncentracijos buvo reikšmingai didesnės nei kontrolinės grupės vaikams tiek serume, tiek sinoviniame skystyje (p 5785 ng/ml) ir IL-6 (> 5,5 pg/ml) koncentracijos ūminėje artrito stadijoje buvo nustatytos tiems pacientams, kuriems sąnarių liga įgavo lėtinę eigą. Vertinome tai, kaip reikšmingus lėtinės sąnarių ligos kriterijus. Šiame darbe sergančių artritų vaikų funkcinė būklė ir vaiko gyvenimo kokybė pirmą kartą Lietuvoje buvo įvertinta naujai įdiegiamu ne tik Lietuvoje, bet ir pasaulyje, įvairiapusiu jaunatvinių artritų vertinimo klausimynu JAMAR, bei duomenys palyginti su seniau Lietuvoje naudojamu vaiko sveikatos būklės vertinimo klausimynu... [toliau žr. visą tekstą] The earlier studies proved that nearly 70% of children with chronic arthritis reach the adulthood with a certain level of disability and restriction of daily activities. Therefore, it is very important to analyse the causes of arthritis and prognostic factors in the early stages of disease. The aim of our study was to determine whether the changes of laboratory, immunological and interleukins (MRP8/14 (calprotectin), IL-6, IL-33) indexes, assessment of functional condition, and disease activity may predict a chronic arthritis in children. The study showed that MRP8/14 (calprotectin) and Il-6 concentrations in the serum and synovial fluid of children with arthritis were significantly higher than in the control group of healthychildren (p 5785ng/ml) and IL-6 (> 5.5 pg/ml) at the acute stage of arthritis were determined for those patients, whose were developed chronic arthritis. It was evaluated as significant criterion of the chronic joint disease. For the first time in Lithuania, the functional ability and the quality of life in children with arthritis were assessed using a newly introduced multidimensional assessment questionnaire for juvenile arthritis (JAMAR), and data was compared... [to full text]