Burden of comorbid conditions in children and young people with juvenile idiopathic arthritis: a collaborative analysis of 3 JIA registries.

OBJECTIVES: Burden of comorbidities are largely unknown in JIA. From 2000, national and international patient registries were established to monitor biologic treatment, disease activity and adverse events in patients with JIA. The aim of this analysis was to investigate in parallel, for the first time, three of the largest JIA registries in Europe/internationally-UK JIA Biologic Registers (BCRD/BSPAR-ETN), German biologic registers (BiKeR/JuMBO), multinational Pharmachild-to quantify the occurrence of selected comorbidities in patients with JIA. METHODS: Information on which data the registers collect were compared. Patient characteristics and levels of comorbidity were presented, focussing on four key conditions: uveitis, MAS, varicella, and history of tuberculosis. Incidence rates of these on MTX/biologic therapy were determined. RESULTS: 8066 patients were registered into the three JIA registers with similar history of the four comorbidities across the studies; however, varicella vaccination coverage was higher in Germany (56%) vs UK/Pharmachild (16%/13%). At final follow-up, prevalence of varicella infection was lower in Germany (15%) vs UK/Pharmachild (37%/50%). Prevalence of TB (0.1-1.8%) and uveitis (15-19%) was similar across all registers. The proportion of systemic-JIA patients who ever had MAS was lower in Germany (6%) vs UK (15%) and Pharmachild (17%). CONCLUSION: This analysis is the first and largest to investigate the occurrence of four important comorbidities in three JIA registries in Europe and the role of anti-rheumatic drugs. Combined, these three registries represent one of the biggest collection of cases of JIA worldwide and offer a unique setting for future JIA outcome studies.

The Serbian version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR).

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient-reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Serbian language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, test-retest reliability, and construct validity (convergent and discriminant validity). A total of 248 JIA patients (5.2% systemic, 44.3% oligoarticular, 23.8% RF-negative polyarthritis, 26.7% other categories) and 100 healthy children were enrolled in three centres. The JAMAR components discriminated healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. In conclusion, the Serbian version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.

Interleukin-17A Levels Increase in Serum of Children With Juvenile Idiopathic Arthritis.

OBJECTIVES: This study aims to determine the serum levels of interleukin-17A (IL-17A) in children with juvenile idiopathic arthritis (JIA) and analyze the correlation between IL-17A values and disease activity, certain clinical features, and laboratory markers of inflammation. PATIENTS AND METHODS: The study included 30 children (7 boys, 23 girls; mean age 8.8±5.3 years; range 1 to 18 years), who had been diagnosed with JIA (18 children were diagnosed during the study period and 12 children were diagnosed before the start of the study) and had active disease during the study period. Control group included 30 healthy, age- and sex- matched children (9 boys, 21 girls; mean age 8.3±4.8 years; range 1 to 18 years). The enzyme-linked immunosorbent assay was used to assess the serum IL-17A levels of children with JIA in the active phase of the disease and control group. Clinical and laboratory features of the disease were evaluated for the children with JIA. RESULTS: Serum levels of IL-17A in children with JIA were significantly higher in comparison to control group. In children with JIA who were prospectively monitored, statistically significantly decreased IL-17A level was recorded in the inactive phase of the disease. The incidence of arthritis of coxofemoral joints was significantly more common, and the mean levels of erythrocyte sedimentation rate and C-reactive protein were significantly higher in the group of children with JIA with detectable levels of IL-17A. Children with JIA and detectable levels of IL-17A had significantly higher values of Juvenile Arthritis Disease Activity Score-27 in comparison to children with JIA and non-detectable IL-17A. CONCLUSION: Assessment of serum IL-17A levels in early phases of JIA gives an opportunity for early detection of children that have higher risk for worse functional outcome.

Dystrophic calcifications and Raynaud's phenomenon in an eight-year old girl.

INTRODUCTION: Dystrophic calcifications are the most common subtype of skin calcinosis. Tumorous soft tissue calcium deposits usually contain hydroxyapatite and amorphous calcium phosphate. Differential diagnosis of skin calcinosis encompasses Thibierge-Weissenbach syndrome, systemic sclerosis, scleroderma, CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia), dermatomyositis, systemic lupus erythematosus, ad myositis ossificans progressiva. CASE OUTLINE: We present the case of an eight-year old girl with tumorous soft tissue calcium deposits and Raynaud's phenomenon. At the age of 3.5 years, our patient was admitted to Pediatric Surgery Clinic because of bilateral acrocyanosis localized at the fingertips area of hands, with the signs of vascular trauma. Therapy with vasodilators and hyperbaric oxygen treatment were completed. This therapy resulted in improvement. At the age of eight, the patient was admitted again due to intermittent, painful cramps localized in both hands. Punctiform deposits were present at the tips of fingers and toes, which looked like calcifications and were spontaneously eliminated, with the remnants of crater-shaped defects. A hard tumorous deformity localized in soft tissue was present in the extensor area of the right elbow. Laboratory indicators of inflammation were within the reference values, and antinuclear antibodies were positive. A nodus localized at the right elbow was extirpated. Pathohistological findings: connective and fat tissue with large deposits of calcium. CONCLUSION: Further follow-up of our patient is necessary due to possible development of complete picture of CREST syndrome or systemic sclerosis.

[Allergic complications of L-asparaginase therapy in children with acute lymphoblastic leukaemia].

INTRODUCTION: L-asparaginase (L-ASP) is one of the most effective medications for the treatment of acute lymphoblastic leukaemia (ALL) in children, and allergic reactions to the therapy are considered the most significant side effects. OBJECTIVE: The aim of this study was to determine the prevalence and type of allergic reactions, as well as to identify potential risk factors for the development of allergic reactions during L-ASP therapy in children with ALL. METHODS: The study encompassed 70 patients under 18 years of age, who were treated at the Institute for Child and Youth Healthcare of Vojvodina, Novi Sad in the period January 2000 - June 2009. We analyzed the frequency and type of allergic reactions during the administration of L-ASP, the onset of allergic reaction in relation to the phase of therapy of underlying disease, as well as the prevalence of allergic reactions in relation to drug administration method. RESULTS: Allergic reaction manifested in 17 patients (24%). In 14 patients (82%) allergic reaction to L-ASP manifested as urticaria, bronchospasm or anaphylaxis, whereas a mild local reaction was observed in only three patients (18%). In a group treated, according to the high-risk protocol, the prevalence of allergic reactions was statistically significantly higher in the intermediate-risk group of patients (p < 0.01), i.e. statistically significantly more frequent, as compared to the standard-risk group of patients (p < 0.05). The majority of patients (11; 65%) developed allergic reactions to the 9th dose of L-ASP, i.e. the first dose during the reinduction phase. The time interval between the last L-ASP dose in the induction phase and the 1st dose in the reinduction phase was at least four weeks. With respect to administration method, the majority of patients (16; 94%) developed allergic reaction after intravenous application of L-ASP. CONCLUSION: Potential risk factors for the development of allergic reaction to L-ASP are a high-risk therapy group, intravenous administration route and repeated application of the drug after at least four-week cessation period.