Bianca A. Lang

Early predictors of poor functional outcome in systemic‐onset juvenile rheumatoid arthritis: A multicenter cohort study

OBJECTIVEnTo examine the ability of a previously described set of criteria to predict poor functional outcome in a large, multicenter cohort of children with systemic-onset juvenile rheumatoid arthritis (JRA).nnnMETHODSnAll children who were diagnosed with systemic-onset JRA since 1980 at the Hospital for Sick Children (Toronto), since 1983 at the Isaac Walton Killam Hospital for Children (Halifax), and since 1981 at the Childrens Hospital of Eastern Ontario (Ottawa) were evaluated. Patients were included in the study if they had been evaluated clinically within 6 months of diagnosis and had been followed up for at least 2 years. Patients were divided into 4 cohorts according to their length of followup: 2-4 years, 4-7 years, 7-10 years, and >10 years. Using previously described criteria for destructive arthritis in children with systemic-onset JRA, the patients were classified as either high risk or low risk for poor functional outcome based on the data from their 6-month visit. High-risk patients had active systemic disease (persistent fever or corticosteroid requirement for control of systemic disease) and a platelet count > or =600 x 10(9)/liter. Poor outcome was defined as moderate or severe disability (defined as a score of > or =0.75 on the Childhood Health Assessment Questionnaire) or disease-associated death.nnnRESULTSnAmong 122 eligible patients with systemic-onset JRA, we were able to contact 111 (91%) for outcome data. The mean followup period was 7.7 years (SD 3.7). The mean age at outcome assessment was 13.5 years (SD 5.3). There were 51 boys and 60 girls. Twenty-four patients (22%) had moderate-to-severe disability and 2 patients died; these 26 patients were considered to have had a poor outcome. We could determine risk classification for 104 patients. Twenty-four patients (23%) met the criteria for high risk at the 6-month visit. Overall, the risk of a poor functional outcome was significantly higher in the high-risk group (relative risk 3.3, 95% confidence interval [95% CI] 1.73-6.43, P = 0.0004). This risk was most marked in the cohort with > 10 years of followup (relative risk 4.3, 95% CI 1.82-10.29, P = 0.006).nnnCONCLUSIONnThe presence of active systemic disease at 6 months, as characterized by fever or the need for corticosteroids, and thrombocytosis strongly predicted the development of a poor functional outcome in these patients. This was especially apparent with longterm followup. Our study validates the previously developed prognostic criteria for systemic-onset JRA.

Parent-child interactions among children with juvenile fibromyalgia, arthritis, and healthy controls.

&NA; Parent–child interactions during pain‐inducing exercise tasks among children (11–17 years old) with fibromyalgia, juvenile rheumatoid arthritis, and pain‐free controls were examined and the contribution of parent–child interactions to disability was tested. Fifteen children in each of the three diagnostic groups and their parents completed 5‐min exercise tasks and completed questionnaire measures of disability (Functional Disability Inventory) and coping (Pain Coping Questionnaire). There were few group differences in parent–child interactions. After controlling for childrens ratings of pain evoked by the exercise, group differences in interactions during exercise tasks were no longer significant. Sequential analyses, controlling for group and exercise task, revealed that when parents made statements discouraging coping following childrens negative verbalizations about the task or pain, children were less likely to be on task, compared to when parents made statements encouraging coping or when parents made any other statements. Childrens general pain coping strategies were not related to parent–child interactions. Parent–child interactions were generally not related to disability. Across the groups, more pain and less time on task during the exercises were related to Functional Disability Inventory scores and more school absences. Parent–child interaction patterns influence childrens adaptation to pain during experimental tasks. Parents discouragement of coping in response to their childrens negative statements related to the pain or the pain‐evoking task are counter productive to childrens ability to maintain activity in a mildly painful situation.

Seasonal onset of systemic-onset juvenile rheumatoid arthritis☆☆☆★

OBJECTIVEnThis study was undertaken to investigate the recent finding of a seasonal difference in the onset of systemic-onset juvenile rheumatoid arthritis (SoJRA). We hypothesized that a seasonal onset pattern might implicate on infectious agent as a cause of SoJRA.nnnMETHODSnThe date of onset was collected from the records of all patients with SoJRA from 1980 to 1992 at presentation to pediatric rheumatology clinics across Canada. The onset pattern of SoJRA was then compared with incidence data on viral infections obtained for the same period.nnnRESULTSnAcross Canada the onset of SoJRA was constant across the seasons. However, in the Prairie region there was a statistically significant seasonal pattern, with peaks in autumn and early spring. We could find no evidence that viral incidence correlated with disease incidence either throughout Canada or in the Prairie region.nnnCONCLUSIONSnIf a seasonal infectious agent causes SoJRA, then it is likely only one of several causes and may act only in certain regions. Future studies should be carried out in those areas where SoJRA does have a seasonal onset pattern.

Serum-soluble interleukin-2 receptor levels in Kawasaki disease.

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2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups

Objective To develop and validate new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIM) and their major subgroups. Methods Candidate variables were assembled from published criteria and expert opinion using consensus methodology. Data were collected from 47 rheumatology, dermatology, neurology and paediatric clinics worldwide. Several statistical methods were used to derive the classification criteria. Results Based on data from 976 IIM patients (74% adults; 26% children) and 624 non-IIM patients with mimicking conditions (82% adults; 18% children), new criteria were derived. Each item is assigned a weighted score. The total score corresponds to a probability of having IIM. Subclassification is performed using a classification tree. A probability cut-off of 55%, corresponding to a score of 5.5 (6.7 with muscle biopsy) ‘probable IIM’, had best sensitivity/specificity (87%/82% without biopsies, 93%/88% with biopsies) and is recommended as a minimum to classify a patient as having IIM. A probability of ≥90%, corresponding to a score of ≥7.5 (≥8.7 with muscle biopsy), corresponds to ‘definite IIM’. A probability of <50%, corresponding to a score of <5.3 (<6.5 with muscle biopsy), rules out IIM, leaving a probability of ≥50u2009to <55% as ‘possible IIM’. Conclusions The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for IIM have been endorsed by international rheumatology, dermatology, neurology and paediatric groups. They employ easily accessible and operationally defined elements, and have been partially validated. They allow classification of ‘definite’, ‘probable’ and ‘possible’ IIM, in addition to the major subgroups of IIM, including juvenile IIM. They generally perform better than existing criteria.

2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups

To develop and validate new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIM) and their major subgroups.

EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups: a methodology report

Objective To describe the methodology used to develop new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIMs) and their major subgroups. Methods An international, multidisciplinary group of myositis experts produced a set of 93 potentially relevant variables to be tested for inclusion in the criteria. Rheumatology, dermatology, neurology and paediatric clinics worldwide collected data on 976 IIM cases (74% adults, 26% children) and 624 non-IIM comparator cases with mimicking conditions (82% adults, 18% children). The participating clinicians classified each case as IIM or non-IIM. Generally, the classification of any given patient was based on few variables, leaving remaining variables unmeasured. We investigated the strength of the association between all variables and between these and the disease status as determined by the physician. We considered three approaches: (1) a probability-score approach, (2) a sum-of-items approach criteria and (3) a classification-tree approach. Results The approaches yielded several candidate models that were scrutinised with respect to statistical performance and clinical relevance. The probability-score approach showed superior statistical performance and clinical practicability and was therefore preferred over the others. We developed a classification tree for subclassification of patients with IIM. A calculator for electronic devices, such as computers and smartphones, facilitates the use of the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria. Conclusions The new EULAR/ACR classification criteria provide a patient’s probability of having IIM for use in clinical and research settings. The probability is based on a score obtained by summing the weights associated with a set of criteria items.

New Onset Juvenile Dermatomyositis (JDMS): Comparisons with a healthy cohort and children with juvenile rheumatoid arthritis. |[bull]| 1845

To determine in a case-control study if new onset JDMS patients had increased 1) symptoms, 2)environmental conditions, 3) familial autoimmune diseases, or 4) antibody titers, compared with two control groups. The families of 80 JDMS and 62 JRA or healthy children had a structured interview. All childrens sera were tested for antibody to Toxoplasma gondii, Herpes simplex virus (HSV), or Coxsackievirus B (CVB). Children with JDMS had symptoms 3 months before disease index date, p=0.013. JRA families had more rheumatoid arthritis (p=.0001) and pernicious anemia (p=.003) than those of JDMS or healthy children. Enteroviral titers were higher in JDMS children ≤ 7 years of age (84%) and controls (89%) than JRA (53%), suggesting an environmental exposure. Titers to T. gondii, HSV or CVB 1-6 were normal. Conclusion: Familial autoimmune disease, environmental factors, or antibody titers to T. gondii, HSV or CVB are not increased in JDMS. JDMS children have symptoms three months before disease index date; young patients have elevated enteroviral titers as do regional controls.

Juvenile Dermatomyositis at Onset: Demographics, Clinical Characteristics, and Access to Care. ♦ 1844

Juvenile Dermatomyositis at Onset: Demographics, Clinical Characteristics, and Access to Care. ♦ 1844