Kristin Houghton

Incident vertebral fractures among children with rheumatic disorders 12 months after glucocorticoid initiation: a national observational study.

To determine the frequency of incident vertebral fractures (IVF) 12 months after glucocorticoid (GC) initiation in children with rheumatic diseases and to identify children at higher risk.

The outcomes of juvenile idiopathic arthritis in children managed with contemporary treatments: results from the ReACCh-Out cohort

Objective To describe clinical outcomes of juvenile idiopathic arthritis (JIA) in a prospective inception cohort of children managed with contemporary treatments. Methods Children newly diagnosed with JIA at 16 Canadian paediatric rheumatology centres from 2005 to 2010 were included. Kaplan–Meier survival curves for each JIA category were used to estimate probability of ever attaining an active joint count of 0, inactive disease (no active joints, no extraarticular manifestations and a physician global assessment of disease activity <10 mm), disease remission (inactive disease >12 months after discontinuing treatment) and of receiving specific treatments. Results In a cohort of 1104 children, the probabilities of attaining an active joint count of 0 exceeded 78% within 2 years in all JIA categories. The probability of attaining inactive disease exceeded 70% within 2 years in all categories, except for RF-positive polyarthritis (48%). The probability of discontinuing treatment at least once was 67% within 5 years. The probability of attaining remission within 5 years was 46–57% across JIA categories except for polyarthritis (0% RF-positive, 14% RF-negative). Initial treatment included joint injections and non-steroidal anti-inflammatory drugs for oligoarthritis, disease-modifying antirheumatic drugs (DMARDs) for polyarthritis and systemic corticosteroids for systemic JIA. Conclusions Most children with JIA managed with contemporary treatments attain inactive disease within 2 years of diagnosis and many are able to discontinue treatment. The probability of attaining remission within 5 years of diagnosis is about 50%, except for children with polyarthritis.

Early outcomes and improvement of patients with juvenile idiopathic arthritis enrolled in a Canadian multicenter inception cohort

To determine early outcomes and early improvements in a prospective inception cohort of children with juvenile idiopathic arthritis (JIA) treated with current standard therapies.

Fitness, fatigue, disease activity, and quality of life in pediatric lupus.

OBJECTIVE To measure aerobic fitness (maximum oxygen consumption [VO(2peak)]), fatigue, quality of life (QOL), and disease activity in young persons with systemic lupus erythematosus (SLE), and to determine an equation for predicting VO(2peak) from the distance walked in 6 minutes (6MW). METHODS Fifteen young patients ages 12-19 years with SLE participated. VO(2) was measured by a graded treadmill exercise test. Submaximal exercise intensity was determined from the ventilatory anaerobic threshold. Submaximal aerobic capacity was measured using the 6MW. Patient questionnaires included measures of fatigue, QOL, and physical activity. Physician questionnaires included the Systemic Lupus Erythematosus Disease Activity Index and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index. RESULTS Compared with age-matched norms, our patients had moderate impairment in aerobic fitness, with a mean +/- SD VO(2peak) of 31.1 +/- 7.9 ml/minute/kg and a mean 6MW distance Z score of -2.4 +/- 2.3. The regression equation to predict VO(2peak) (ml/minute/kg) from 6MW was as follows: 57.1 + [0.038 x distance (meters)] + (-0.35 x maximal heart rate) (R = 0.67, P = 0.027). Ten subjects (67%) reported significant fatigue. There was no significant correlation of fatigue with fitness measures. Neither fatigue nor fitness was significantly correlated with disease activity, disease damage, or QOL measures. CONCLUSION Young SLE patients have lower aerobic fitness than reference norms. The 6MW may be used as a marker of fitness, but it is preferable to determine VO(2) with a graded exercise test. Fatigue is a significant symptom in young SLE patients. The application of fatigue measures in young persons is exploratory. The relationship between fatigue and aerobic fitness is not clear.

Predictors of early inactive disease in a juvenile idiopathic arthritis cohort: Results of a Canadian multicenter, prospective inception cohort study

OBJECTIVE To determine early predictors of 6-month outcomes in a prospective cohort of patients with juvenile idiopathic arthritis (JIA). METHODS Patients selected were those enrolled in an inception cohort study of JIA, the Research in Arthritis in Canadian Children Emphasizing Outcomes Study, within 6 months after diagnosis. The juvenile rheumatoid arthritis core criteria set and quality of life measures were collected at enrollment and 6 months later. Outcomes evaluated included inactive disease, Juvenile Arthritis Quality of Life Questionnaire (JAQQ) scores, and Childhood Health Assessment Questionnaire (C-HAQ) scores at 6 months. RESULTS Thirty-three percent of patients had inactive disease at 6 months. Onset subtype and most baseline core criteria set measures correlated with all 3 outcomes. Relative to oligoarticular JIA, the risks of inactive disease were lower for enthesitis-related arthritis, polyarthritis rheumatoid factor (RF)-negative JIA, and polyarthritis RF-positive JIA, and were similar for psoriatic arthritis. In multiple regression analyses, the baseline JAQQ score was an independent predictor of all 3 outcomes. Other independent baseline predictors included polyarthritis RF-negative and systemic JIA for inactive disease; C-HAQ score and polyarthritis RF-positive JIA for the 6-month C-HAQ score; and active joint count, pain, and time to diagnosis for the 6-month JAQQ score. CONCLUSION Clinical measures soon after diagnosis predict short-term outcomes for patients with JIA. The JAQQ is a predictor of multiple outcomes. Time to diagnosis affects quality of life in the short term.

Incident vertebral fractures and risk factors in the first three years following glucocorticoid initiation among pediatric patients with rheumatic disorders

Vertebral fractures are an important yet underrecognized manifestation of osteoporosis in children with chronic, glucocorticoid‐treated illnesses. Our goal was to determine the incidence and clinical predictors of vertebral fractures in the 3 years following glucocorticoid initiation among pediatric patients with rheumatic disorders. Incident vertebral fractures were evaluated according to the Genant semiquantitative method on lateral radiographs at baseline and then annually in the 3 years following glucocorticoid initiation. Extended Cox models were used to assess the association between vertebral fractures and clinical risk predictors. A total of 134 children with rheumatic disorders were enrolled in the study (mean ± standard deviation (SD) age 9.9 ± 4.4 years; 65% girls). The unadjusted vertebral fracture incidence rate was 4.4 per 100 person‐years, with a 3‐year incidence proportion of 12.4%. The highest annual incidence occurred in the first year (6.0%; 95% confidence interval (CI) 2.9% to 11.7%). Almost one‐half of the patients with fractures were asymptomatic. Every 0.5 mg/kg increase in average daily glucocorticoid (prednisone equivalents) dose was associated with a twofold increased fracture risk (hazard ratio (HR) 2.0; 95% CI 1.1 to 3.5). Other predictors of increased vertebral fracture risk included: (1) increases in disease severity scores between baseline and 12 months; (2) increases in body mass index Z‐scores in the first 6 months of each 12‐month period preceding the annual fracture assessment; and (3) decreases in lumbar spine bone mineral density Z‐scores in the first 6 months of glucocorticoid therapy. As such, we observed that a clinically significant number of children with rheumatic disorders developed incident vertebral fractures in the 3 years following glucocorticoid initiation. Almost one‐half of the children were asymptomatic and thereby would have been undiagnosed in the absence of radiographic monitoring. In addition, discrete clinical predictors of incident vertebral fractures were evident early in the course of glucocorticoid therapy.

Review for the generalist: evaluation of pediatric hip pain

Hip pathology may cause groin pain, referred thigh or knee pain, refusal to bear weight or altered gait in the absence of pain. A young child with an irritable hip poses a diagnostic challenge. Transient synovitis, one of the most common causes of hip pain in children, must be differentiated from septic arthritis. Hip pain may be caused by conditions unique to the growing pediatric skeleton including Perthes disease, slipped capital femoral epiphysis and apophyseal avulsion fractures of the pelvis. Hip pain may also be referred from low back or pelvic pathology. Evaluation and management requires a thorough history and physical exam, and understanding of the pediatric skeleton. This article will review common causes of hip and pelvic musculoskeletal pain in the pediatric population.

Review for the generalist: evaluation of low back pain in children and adolescents

Back pain is common in children and adolescents. Most cases of back pain are non-specific and self-limiting. In children and adolescents, pain is usually related to the posterior elements of the spine and disc-related problems are rare. Serious pathology, including malignancy and infection needs to be excluded. Evaluation and management is challenging and requires a thorough history and physical exam, and understanding of the immature skeleton. Diagnostic imaging is useful in the evaluation of a child or adolescent with low back pain and can help guide management. This article will review common causes of back pain in the pediatric population.

Rituximab Treatment in a Child with Rosai-Dorfman Disease and Systemic Lupus Erythematosus

To the Editor: Rosai-Dorfman disease (RDD) is a rare disorder in childhood. The association of RDD and systemic lupus erythematosus (SLE) is very rare, with only 2 previously reported cases, and neither of these in a child1,2. We describe a child who presented with histopathology-confirmed RDD, and developed SLE 3 years later. A 7-year-old boy of Canadian First Nations background presented at age 4 years to the pediatric hematology/oncology clinic with a 3-month history of asymptomatic, bilateral massive lymphadenopathy involving the cervical, supraclavicular, mediastinal, hilar, and axillary lymph nodes. He has a healthy fraternal twin, and there is no family history of connective tissue diseases. The lymphadenopathy failed to respond to a course of antibiotics. Laboratory tests showed mild microcytic hypochromic anemia with lymphopenia [white blood cells (WBC) 6.29 x 109/l, hemoglobin 103 g/l, mean corpuscular volume 74.3 fl, mean corpuscular hemoglobin 23.5 pg/cell, platelets 262 x 109/l, lymphocytes 0.63 x 109/l, absolute neutrophil count 4.98 x 109/l) and high erythrocyte sedimentation rate, 63 mm/h. Serial chest radiographs showed persistent nonprogressive large bilateral mediastinal, hilar, right infrahilar, and paratracheal lymphadenopathy (Figure 1). Computed tomography scan showed adenopathy, airway narrowing, and atelectasis as well as superior vena cava displacement (Figure … Address correspondence to Dr. Alqanatish; E-mail: jqanatish{at}hotmail.com

Development of System-level Performance Measures for Evaluation of Models of Care for Inflammatory Arthritis in Canada

Objective. To develop system-level performance measures for evaluating the care of patients with inflammatory arthritis (IA), including rheumatoid arthritis (RA), psoriatic arthritis, ankylosing spondylitis, and juvenile idiopathic arthritis. Methods. This study involved several methodological phases. Over multiple rounds, various participants were asked to help define a set of candidate measurement themes. A systematic search was conducted of existing guidelines and measures. A set of 6 performance measures was defined and presented to 50 people, including patients with IA, rheumatologists, allied health professionals, and researchers using a 3-round, online, modified Delphi process. Participants rated the validity, feasibility, relevance, and likelihood of use of the measures. Measures with median ratings ≥ 7 for validity and relevance were included in the final set. Results. Six performance measures were developed evaluating the following aspects of care, with each measure being applied separately for each type of IA except where specified: waiting times for rheumatology consultation for patients with new onset IA, percentage of patients with IA seen by a rheumatologist, percentage of patients with IA seen in yearly followup by a rheumatologist, percentage of patients with RA treated with a disease-modifying antirheumatic drug (DMARD), time to DMARD therapy in RA, and number of rheumatologists per capita. Conclusion. The first set of system-level performance measures for IA care in Canada has been developed with broad input. The measures focus on timely access to care and initiation of appropriate treatment for patients with IA, and are likely to be of interest to other arthritis care systems internationally.