S Yokota

Prognostic factors in influenza-associated encephalopathy.

Background: Recently, reports of influenza-associated encephalopathy have increased worldwide. Given the high mortality and morbidity rates attributable to this severe neurologic complication of influenza, we conducted a nationwide study in Japan to identify the prognostic factors. Methods: We retrospectively evaluated 442 cases of influenza-associated encephalopathy that were reported to the Collaborative Study Group on Influenza-Associated Encephalopathy, which was organized by the Japanese Ministry of Health, Labor, and Welfare in collaboration with hospitals, clinics, and local pediatric practices in Japan between 1998 and 2002. The outcome for each patient was classified as either survival or death. Predictors of death were identified using logistic regression analysis. Results: Four major prognostic factors for death were found to be significant by multivariate analysis (P < 0.05) in the 184 patients for whom we had complete data: elevation of aspartate aminotransferase, hyperglycemia, the presence of hematuria or proteinuria, and use of diclofenac sodium. Conclusions: We identified patients who had factors associated with a poor prognosis, and these findings might be clinically useful for the management of this illness.

2.1 Long-term safety and efficacy of tocilizumab in children with systemic juvenile idiopathic arthritis (JIA)

Results Sixty seven patients (29 boys and 38 girls) were included in this analysis. Median age was 8 years and median disease duration was 3.8 years. At the time of analysis, 9 patients had discontinued tocilizumab treatment, 4 due to AEs, 4 due to development of anti-tocilizumab antibodies and 1 due to lack of efficacy. Median duration of tocilizumab treatment was 146 weeks. The most frequent AEs were upper respiratory tract infections and gastroenteritis. The incidence rate of serious infections was 10.7 per 100 patient-years. No deaths, malignancies, or autoimmune diseases were observed. ACR Pedi 30, 50 and 70 Improvement Criteria were achieved in 100%, 98% and 93% at Week 96. All patients were treated with oral corticosteroids at the registration and 72% were able to reduce corticosteroid dose by more than 50% at Week 96. Fourteen patients became steroid-free during the study. 4 patients were in remission without tocilizumab itself and any other medications.

PReS-FINAL-2189: Tocillizumab for patients with takayasu arteritis in childhood refractory to conventional therapy

Takayasu arteritis (TA) is a chronic systemic granulomatous vasculitis of large vessels. Corticosteroids may induce remission. However, over half of patients flare with tapering of corticosteroids. There are anecdotal reports of treatment with methotrexate, azathioprine, and biologics such as infliximab. Cyclophosphamide is recommended for life-threatening or organ-threatening patients. However, patients, especially with HLA B52-positive, are frequently refractory to these treatments. Since serum levels of interleukin (IL)-6 are correlated with disease activity, an open-label trial of tocilizumab (TCZ), an anti-IL-6 receptor monoclonal antibody, was conducted for 6 pediatric patients with TA.

PReS-FINAL-2166: Long-term safety and effectiveness of anti-interleukin-6 receptor monoclonal antibody, tocilizumab, in patients with systemic juvenile idiopathic arthritis in Japan

Systemic-onset juvenile idiopathic arthritis (sjia) is a form of childhood chronic arthritis of unknown etiology with systemic manifestations such as remittent fever and erythematous rash, lymph adenopathy, hepatosplenomegaly, and/or serositis. Tocilizumab (TCZ) is a humanized anti-IL-6 receptor monoclonal antibody that has been approved for the treatment of patients with sjia. Results of the lead-in phase; the placebo-controlled, double-blind phase; and the first 48 weeks of an open-label extension have been already published.

PW02-024 - A case of candle syndrome treated with thalidomide

A new group of autoinflammatory diseases caused by immunoproteasome dysfunction has been recently reported. The mutation in the PSMB8 gene encoding immunoproteasome subunit β type 8 causes a number of clinical syndromes that described as chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome and Nakajo-Nishimura syndrome (NNS).

Cerebral blood flow in a case of fetal brain death syndrome

H. Werner*†, J. R. Lopes dos Santos‡§, R. Fontes‡, E. L. Gasparetto†¶, P. A. Daltro†, Y. Kuroki† and R. C. Domingues† †Clı́nica de Diagnóstico por Imagem, ‡Instituto Nacional de Tecnologia and ¶Department of Radiology, Federal University of Rio de Janeiro School of Medicine, Rio de Janeiro, Brazil, and §Royal College of Art, London, UK *Correspondence. (e-mail: heron.werner@gmail.com) DOI: 10.1002/uog.6253 Published online 13 November 2008

PReS-FINAL-2172: Efficacy of corticosteroids and intravenous cyclophosphamide for patients with juvenile systemic sclerosis

Systemic sclerosis (ssc) is a rare multisystemic disease characterized by inflammation, vascular abnormalities, and fibrosis that affects the skin and various internal organs. Juvenile ssc accounts for fewer than 10% of all adults with ssc. Regarding effective treatment there were no specific pediatric data available, and the long-term efficacy of treatment for children with ssc has not been investigated.

PReS-FINAL-2110: Tocilizumab for patients with oligoarticular juvenile idiopathic arthritis refractory to conventional therapy

Because most children with oligoarticular juvenile idiopathic arthritis (o-JIA) were mildly affected (Steinblocker functional class I, c.a.85%), o-JIA is tend to be thought of as a mild subtype of JIA. However, 6% of them were unable to participate in a full school program 5 years after diagnosis, and 0.5% of children with o-JIA progressed to class III or IV, severe to terminal stages. Moreover, in 20% of o-JIA patients there is a progressive increase in the number of affected joints after the first 6 months of disease (extended type). These severe patients had never gone into remission despite the conventional therapy.

OP0178 Tocilizumab-effects on growth impairment in systemic juvenile idiopathic arthritis

Background The safety and efficasy of anti-IL-6 receptor monoclonal antibody, tocilizumab (TCZ) has been reported in children with systemic juvenile idiopathic arthritis (s-JIA). Objectives Growth analysis during the study was performed. Methods Forty-five s-JIA patients (8.1±4.2 years) who completed phase-III study of TCZ were enrolled. Mean standard deviation score (SDS) for height, changes in SDS from baseline (ΔSDS), correlation between ΔSDS and several factors such as age, disease duration, corticosteroid dose exposure were evaluated. Yearly height velocity analysis was made with 28 patients who had data for 1 year prior to TCZ administration and received TCZ for more than 1 year. Results Thirty-eight of 45 (84%) obtained clinical response at week 144. The baseline SDS-height was -2.7±2.0 with inverse correlation with disease duration. Significant improvement was seen in height velocity SDS changes from 1 year prior to 1 year posterior to baseline (n=28, -6.0±4.0 to-2.5±3.9, p=0.0064). Reduction in corticosteroid exposure was significantly associated with improvement in height velocity SDS (p=0.0027). Standardized height velocity continued to improve over 3 years of TCZ, whereas average daily prednisolone equivalent dose showed inverse correlation (n=17, Figure). Conclusions Growth impairments evidenced by SDS-height were more prominent in patients with longer standing disease. Catch up growth was observed in patients who required less or no corticosteroid during TCZ treatment. References Yokota S, et al. Lancet. 2008 Mar 22;371(9617):998-1006. Disclosure of Interest None Declared

SAT0407 Association between the rates of enlarged nailfold capillaries and serum FDP-E levels reflects microvascular injuries in juvenile dermatomyositis

Background One indicator of the active microvascular damages of juvenile dermatomyositis (JDM) is enlarged nailfold capillary (NFC). We have reported that serum FDP–E level is a marker of fibrinogenolysis and reflects maicroangiopathy in JDM patients. Objectives To determine clinical factors associated with the rates of enlarged NFCs. Methods Forty NFC images of fifteen JDM patients (7.3±3.9 years at first visit) were evaluated. NFC findings of 4 fingers of hand, excluding thumb were recorded in maintaining a constant room temperature of range 24–26°C. The rates of enlarged capillaries (>20 μm) to total number of end raw loops per millimeter in 4 digits were evaluated. Correlations between the rates of enlarged NFCs and serum level of CK, aldorase, FDP-E and ESR were examined. Associations between clinical manifestations (skin involvemrnts and muscle weakness) and the rates of enlarged NFCs were also evaluated. The rates of enlarged NFCs were devided into four groups in this study (group 1=0∼25%, group 2=26∼50%, group 3=51∼75%, group 4=76∼100%), and rates of abnormal levels of serum CK, aldolase, FDP-E, and ESR were evaluated, respectively. Results The rates of enlarged NFCs were significantly associated with serum FDP-E levels (r=0.34, P<0.05). Skin involvements were associated with higher rates of enlarged NFCs. Furthermore, abnormal FDP-E levels were found in 33% in group 1, whereas in group 4 abnormal FDP-E levels were found in all patients (p=0.0034). Conclusions Enlarged NFCs may reflect microangiopathy in active disease of JDM. Serum FDP-E levels will indicate microvascular inflammation especially in skin involvements. References Takayuki K, et al. Clinical analysis of juvenile dermatomyositis; a retrospective study of 45 Japanese. Eular congress. 2009 Disclosure of Interest None Declared