New advances in the management of juvenile idiopathic arthritis--1: non-biological therapy.

Juvenile idiopathic arthritis (JIA) is the most common paediatric rheumatic disease with significant long-term morbidity and mortality. Major advances have taken place in recent years in our understanding and the evidence base of JIA. Key to this has been the serious collaborative efforts of clinicians, academics, scientists and the whole of the multidisciplinary team. This has led to the important recognition and development of specialised expertise for the management of patients, improvement in global outcome measures and aggressive treatment of the significant complications of JIA. Important steps have taken place in optimising treatment of JIA. Clinical trials demonstrate that early use of intra-articular corticosteroid injections alone or in addition to other systemic treatments can have a long-lasting effect. Robust evidence has defined the importance of methotrexate as the first-line disease modifying anti-rheumatic drug in JIA. Newer treatment options in severe refractory disease are now available including stem cell transplantation. This review focuses on the recent advances in non-biological therapies for treating JIA.

New advances in the management of juvenile idiopathic arthritis--2: the era of biologicals.

Juvenile idiopathic arthritis (JIA) is the most common paediatric rheumatic disease with significant long-term morbidity and mortality. Major advances have taken place in recent years in our understanding and the evidence base of JIA. The advent of biological therapies has opened a major new era in the medical management of JIA with recent trials published of etanercept, infliximab, adalimumab, abatacept, tocilizumab and anakinra. National and international collaborative clinical and research networks are ideally placed to enable future advances in the management of JIA and all paediatric rheumatic disorders. This review follows on from Part 1 of a review of recent advances in non-biological therapies in JIA, and focuses on the significant new advances in biological therapies in managing JIA.

Association between duration of symptoms and severity of disease at first presentation to paediatric rheumatology: results from the Childhood Arthritis Prospective Study.

OBJECTIVES: To study the association between disease severity at first presentation to paediatric rheumatology (PRh) and length of time since symptom onset in children recruited to the Childhood Arthritis Prospective Study. METHODS: Children or=2 weeks were recruited from five UK hospitals. Data including demographics, disease features, Childhood Health Assessment Questionnaire (CHAQ), physician and parent global assessment and blood tests were collected at the first appointment with PRh (baseline). The association between symptom duration (defined as time from first reported symptom onset to presentation at PRh) and baseline disease characteristics was evaluated using non-parametric descriptive statistics and multivariable logistic regression analyses. RESULTS: Five hundred and seven children (65% female) were included: median age at onset was 6.8 yrs. Two hundred and thirty-three had oligoarthritis, 68 had RF-negative polyarthritis, 27 had systemic onset arthritis and 29 had arthritis that was not JIA. The median symptom duration was 4.6 months. Median symptom duration was shortest for children presenting with systemic arthritis (1.6 months) and longest for those with PsA (8.6 months). Children with a longer duration of symptoms were older and had higher median active joint counts but lower median ESR. Symptom duration did not correlate with CHAQ score at presentation. CONCLUSIONS: Children who have systemic arthritis had the shortest delay to PRh presumably because they are profoundly unwell. Children with joint pain/stiffness but normal ESR had longer delays suggesting that if blood tests do not indicate inflammation, the diagnosis of JIA may be overlooked.

Costing juvenile idiopathic arthritis: examining patient-based costs during the first year after diagnosis.

OBJECTIVES: There are few data on the treatment patterns and associated cost of treating children with inflammatory arthritis including juvenile idiopathic arthritis (JIA), in the short or long term. The aim of this study was to obtain patient-based costs for treating children with JIA in the UK, in the first year from diagnosis and from the secondary health care payer perspective. METHODS: The Childhood Arthritis Prospective Study (CAPS) is an ongoing longitudinal study recruiting children with inflammatory arthritis from four UK hospital centres. Included children are newly diagnosed,

Treatment approaches to juvenile dermatomyositis.

Juvenile dermatomyositis (JDM) is a rare disease of childhood with significant morbidity. Although the mortality and morbidity has improved over recent decades, it is still a chronic disease for a significant proportion of children with JDM. There is a paucity of controlled trials in both adult and juvenile dermatomyositis. In this article, the different therapeutic agents used in clinical practice are outlined. Furthermore, our approach to the treatment of JDM with methotrexate and aggressively tapered steroids in an algorithmic fashion, is discussed.

Use of methotrexate in juvenile idiopathic arthritis.

Methotrexate (MTX) has transformed the outlook for children with juvenile idiopathic arthritis (JIA). Most of the evidence from uncontrolled clinical trials suggests that MTX is an effective agent for treating active JIA. Data from controlled clinical trials suggests that MTX has statistically significant effects on patient centred disability measures in JIA patients with active arthritis. Although we would like a much larger study directed evidence base for our use of the drug, the studies that have been done are sound and have been followed by a change in clinical expectations and advice that speak of qualitative evidence from clinical practice, confirming the scientifically acquired data. Randomised controlled multicentre trials using sufficient numbers of patients, including functional assessment and quality of life measures, are needed to confirm the long term efficacy and safety of MTX in JIA.

Anterior knee pain: a long-term follow-up.

OBJECTIVE: To assess the long-term outcome of anterior knee pain diagnosed in childhood. METHOD: Forty-eight patients diagnosed with anterior knee pain in childhood were contacted by postal questionnaire 4-18 yr after initial presentation. RESULTS: The 22 respondents comprised 16 women and six men, with mean age at follow-up of 22 yr. Twenty of 22 (91%) still had knee pains, eight daily, two weekly and 10 occasionally. In 10 (45%) the pain affected their daily life and in eight (36%) it restricted their physical activities. Twelve (54%) used painkillers. Ten (45%) had developed other diagnoses: four had psoriasis and six arthritis, of whom one had ankylosing spondylitis. Fifteen (68%) had symptoms in other joints at follow-up. CONCLUSION: These results suggest that anterior knee pain that occurs in childhood may not be so benign a condition as thought.

Measuring the nutritional status of children with juvenile idiopathic arthritis using the bioelectrical impedance method.

OBJECTIVE: To assess the nutritional status of children with juvenile idiopathic arthritis (JIA) using anthropometric measurements and bioelectrical impedance. METHODS: Twenty-two consecutive JIA patients (seven pauciarticular, 15 polyarticular) attending the rheumatology clinic at Booth Hall Children's Hospital were compared with 22 age- and sex-matched controls attending the accident and emergency department of the same hospital. There were no patients with systemic-onset JIA in the cohort. Height, weight, head circumference and skinfold thickness at four sites (biceps, triceps, subscapular and suprailiac) were measured. Regression equations were used to calculate body fat as a percentage of weight, and arm muscle circumference. In addition, bioelectrical impedance measurements were made using a Holtain body composition analyser. These measurements were then used to calculate the total body water, which could be used as an indirect estimate of the lean body mass. RESULTS: Of the JIA patients, 22.7% were below the third centile for height, 18.1% had a weight less than the third centile. Mid-arm circumference was below the fifth centile in 36.4% of the patients. Patients with polyarticular disease showed significantly more signs of malnutrition than patients with pauciarticular disease. In the polyarticular group, comparison with controls revealed significant P values for reduction in height (0.047), weight (0.045), mid-arm circumference (0.002), arm muscle circumference (0.012), percentage body fat (0.008) and total body water (0.031). CONCLUSIONS: In view of the findings of lower total body water, indicating lower lean mass, in more nutritionally deprived JIA patients (as deduced by the other physical parameters measured), we conclude that bioelectrical impedance is a useful adjunct to anthropometric measures in assessing nutritional status in JIA.

Nitrous oxide analgesia during intra-articular injection for juvenile idiopathic arthritis.

AIMS: To evaluate the efficacy and safety of nitrous oxide-oxygen for children with juvenile idiopathic arthritis (JIA) undergoing intra-articular corticosteroid injection. METHODS: A total of 55 consecutive patients with JIA undergoing intra-articular corticosteroid injection, using self administered nitrous oxide-oxygen for analgesia were studied. Patient, nurse, and parent pain scores were compared using a 0-10 cm visual analogue scale (VAS) immediately after the procedure. RESULTS: A total of 70 joints were injected in 55 patients (median age 13.54 years). The median pain score for patient, nurse, and parent was 1 (0-10 cm VAS). The mean rank patient score was 2.12, which was greater than the nurse score (1.97), which was greater than the parent score (1.91). These differences were significant. There were no serious adverse events in any patient. CONCLUSIONS: Nitrous oxide-oxygen provides safe and effective analgesia for intra-articular injection in children. In some cases, nurses and parents underestimated pain related to the procedure compared to the child.