Etiology, Risk Factors, and Diagnosis of Back Pain in Children and Adolescents: Evidence- and Consensus-Based Interdisciplinary Recommendations.

Using a structured approach and expert consensus, we developed an evidence-based guideline on the diagnosis of back pain and the treatment of non-specific back pain in children and adolescents. The first part comprises etiology, risk factors, and diagnosis. The second part, published in the same issue, includes treatment and prevention. A comprehensive and systematic literature search was conducted to identify relevant guidelines and studies. Based on the findings of this literature search, recommendations on risk factors and diagnosis were formulated and voted on by experts in a structured consensus-building process. Notable red flags for specific back pain and evidence-based risk factors for non-specific back pain in children and adolescents were identified. Only three evidence-based recommendations could be formulated for causes, red flags, and risk factors for back pain, while two recommendations are based on expert consensus. Regarding diagnostics, eight expert consensus recommendations and one evidence-based recommendation could be provided. Despite the importance of adequate diagnosis for the treatment of back pain in children and adolescents, results of this work confirm the deficit in research investment in this area.

Treatment of Unspecific Back Pain in Children and Adolescents: Results of an Evidence-Based Interdisciplinary Guideline.

Using a structured approach and expert consensus, we developed an evidence-based guideline on the treatment and prevention of non-specific back pain in children and adolescents. A comprehensive and systematic literature search identified relevant guidelines and studies. Based on the findings of this literature search, recommendations on treatment and prevention were formulated and voted on by experts in a structured consensus-building process. Physical therapy (particularly physical activity) and psychotherapy (particularly cognitive behavioral therapy) are recommended for treating pediatric non-specific back pain. Intensive interdisciplinary treatment programs should be provided for chronic and severe pain. Drug therapy should not be applied in children and adolescents. Further research on non-specific back pain in childhood and adolescence is strongly needed to reduce the imbalance between the high burden of non-specific back pain in childhood and adolescence and the low research activity in this field.

Diagnosis and Treatment of Angiography Positive Medium to Large Vessel Childhood Primary Angiitis of Central Nervous System (p-cPACNS): An International Survey.

Childhood Primary Angiitis of Central Nervous System (cPACNS) is rare, but can cause significant damage and result in disability or even death. Because of its rarity, the sometimes acute and variable presentation, limited awareness, and the absence of widely accepted diagnostic and therapeutic standards, cPACNS is a diagnostic and therapeutic challenge. Three subcategories of cPACNS exist, including angiography-positive non-progressive p-cPACNS, angiography-positive progressive p-cPACNS which both affects the medium to large vessels, and angiography-negative small vessel sv-cPACNS. Diagnosis and treatment of cPACNS relies on personal experience, expert opinion and case reports/case series. To collect information on diagnostic and therapeutic approaches to transient and progressive cPACNS, a survey was shared among international clinicians (German Society for Pediatric Rheumatology, the Pediatric Rheumatology European Society, the German speaking "Network Pediatric Stroke," and members of the American College of Rheumatology/CARRA Pediatric Rheumatology list server). Results from this survey will be used to define statements toward a consensus process allowing harmonization of diagnostic and therapeutic approaches and the generation of evidence in a rare condition.

Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey.

Childhood primary angiitis of the Central Nervous System (cPACNS) is a rare autoimmune and inflammatory disease. It can result in significant neuronal damage, neurodevelopmental delay and potentially death. Childhood PACNS is divided into subcategories: angiography-positive p-cPACNS that affects medium and large vessels, and angiography-negative small vessel sv-cPACNS. Due to its rarity, variable clinical representation, and the lack of a diagnostic criteria and therapeutic plans, diagnosis and treatment of cPACNS is challenging and approaches vary. This survey collected information on diagnostic and therapeutic approaches to sv-PACNS. It was shared with international clinician networks, including the German Society for Paediatric Rheumatology, the Paediatric Rheumatology European Society, the "Network Paediatric Stroke," and members of the American College of Rheumatology/CARRA Paediatric Rheumatology list server. This project has shown consensus in numerous diagnostic and therapeutic treatment approaches, highlighting key areas which will be utilised to develop statements in the use of expert consensus meetings to standardise diagnostic and therapeutic approaches in this rare inflammatory disease.

Predictors of poor response to methotrexate in polyarticular-course juvenile idiopathic arthritis: analysis of the PRINTO methotrexate trial.

OBJECTIVES: To determine whether baseline demographic, clinical, articular and laboratory variables predict methotrexate (MTX) poor response in polyarticular-course juvenile idiopathic arthritis. METHODS: Patients newly treated for 6 months with MTX enrolled in the Paediatric Rheumatology International Trials Organization (PRINTO) MTX trial. Bivariate and logistic regression analyses were used to identify baseline predictors of poor response according to the American College of Rheumatology pediatric (ACR-ped) 30 and 70 criteria. RESULTS: In all, 405/563 (71.9%) of patients were women; median age at onset and disease duration were 4.3 and 1.4 years, respectively, with anti-nuclear antibody (ANA) detected in 259/537 (48.2%) patients. With multivariate logistic regression analysis, the most important determinants of ACR-ped 70 non-responders were: disease duration > 1.3 years (OR 1.93), ANA negativity (OR 1.77), Childhood Health Assessment Questionnaire (CHAQ) disability index > 1.125 (OR 1.65) and the presence of right and left wrist activity (OR 1.55). Predictors of ACR-ped 30 non-responders were: ANA negativity (OR 1.92), CHAQ disability index > 1.14 (OR 2.18) and a parent's evaluation of child's overall well-being < or = 4.69 (OR 2.2). CONCLUSION: The subgroup of patients with longer disease duration, ANA negativity, higher disability and presence of wrist activity were significantly associated with a poorer response to a 6-month MTX course.

Exposure to animals and risk of oligoarticular juvenile idiopathic arthritis: a multicenter case-control study.

BACKGROUND: An inverse association between early contact with microbial compounds and respiratory allergies is well established. The protective effect of infant contact with animals was also shown for inflammatory bowel disease (IBD) and systemic lupus erythematosus (SLE). We aimed to test the association between animal contact in infancy and oligoarticular juvenile idiopathic arthritis (OA JIA). METHODS: Parents of children with OA JIA registered at the Hospital for Pediatric Rheumatology in Garmisch-Partenkirchen were asked to complete a questionnaire. Children who underwent strabismus surgery at six referral centers for ophthalmology served as controls. Children age 6 to 18 years born in Germany without malformations were included (238 cases; response 89% and 832 controls; response 86%). Data were analyzed using logistic regression models after adjusting for potential confounders. RESULTS: Neither place of living (urban vs. rural area), living on a farm, nor regular farm animal (adjusted odds ratio 0.79; 95% confidence interval 0.42-1.47) or pet contact (0.79; 0.55-1.14) during infancy were clearly related to case status. Allergic rhinitis was inversely related to OA JIA (0.57; 0.34-0.95).Neither place of living (urban vs. rural area), living on a farm, nor regular farm animal (adjusted odds ratio 0.79; 95% confidence interval 0.42-1.47) or pet contact (0.79; 0.55-1.14) during infancy were related to case status. Allergic rhinitis was inversely related to OA JIA (0.57; 0.34-0.95). CONCLUSIONS: Contact with farm environments in infancy might not be associated with OA JIA. This finding is consistent with previous findings for diabetes mellitus type 1 but contradicts results for IBD and SLE.

Growth hormone increases final height in patients with juvenile idiopathic arthritis: data from a randomized controlled study.

BACKGROUND: GH treatment stimulates growth in short children with juvenile idiopathic arthritis (JIA). The extent to which this therapy increases final height is not known. METHODS: Thirty-one growth-retarded children with systemic and polyarticular idiopathic arthritis were enrolled in this controlled study. After a mean observational time of 8.4 yr, final height was reached in 13 patients (seven females and six males) treated with GH for a mean of 6.7 yr in a dose of 0.33 mg/kg body weight per week. Eighteen patients (12 females and six males) served as an untreated control group. RESULTS: Mean increment in height in the treatment group was 1.6 +/- 0.8 SD, whereas the patients of the control group lost 0.7 +/- 1.8 SD. Overall, mean final height in the treatment group was -1.6 SD and in the control group -3.4 SD. More GH-treated patients reached a final height within target height than untreated patients (11 of 13 vs. four of 18). Disease activity markers had a significant influence on height outcome. After adjustment for baseline and average disease activity, the difference between treatment and control group was still significant (mean 1.5 SD). Patients with a moderate overall disease activity profited most from GH treatment. No adverse events were noted throughout the study. CONCLUSION: Our data suggest that long-term GH therapy has a beneficial effect on growth and final height in the majority of growth retarded children with severe forms of JIA.

Dynamics of body composition and bone in patients with juvenile idiopathic arthritis treated with growth hormone.

INTRODUCTION: GH has a positive impact on growth, bone, and muscle development. The objectives of this study were to demonstrate the effects of GH treatment on regional body composition and bone geometry at final height in patients with juvenile idiopathic arthritis (JIA). PATIENTS AND METHODS: In this longitudinal study, parameters of bone mineral density and geometry as well as muscle and fat cross-sectional area (CSA) in the nondominant forearm were recorded using peripheral quantitative computed tomography at yearly intervals until final height in 12 patients (seven females) receiving GH treatment. Data at final height were compared with 13 patients (nine females) with JIA not treated with GH. RESULTS: Patients were treated with GH for a mean of 5.35 +/- 0.7 yr. Correcting for height, total bone CSA (+0.89 +/- 0.5 sd) and muscle CSA (+1.14 +/- 0.6 sd) increased significantly and normalized at final height. Compared with JIA patients without GH at final height, there was a significantly higher muscle CSA and a lower fat CSA in GH-treated patients. Additionally, in relation to total bone CSA, there was significantly more cortical and less marrow CSA in boys with GH treatment. CONCLUSION: During GH treatment, there was a significant increase and normalization of total bone and muscle CSA at final height. In accordance with an anabolic effect of GH, fat mass stabilized at the lower limit of healthy children. At final height, cortical and marrow CSA, relative to total bone CSA, were normalized in GH-treated patients.

Sonographic investigation of the temporomandibular joint in patients with juvenile idiopathic arthritis: a pilot study.

OBJECTIVE: To evaluate whether there are any correlations between the clinical parameters of temporomandibular joint (TMJ) arthritis and pathologic ultrasound (US) findings of the TMJ in patients with juvenile idiopathic arthritis (JIA). METHODS: We conducted prospective clinical and US investigations of the TMJs of 48 patients with JIA. The US investigation was performed by a 12-MHz high-resolution transducer, which was positioned parallel to the ramus of the mandible overlying the zygomatic arch in a closed-mouth position and maximum open-mouth position. RESULTS: Patients with > or = 5 peripheral affected joints showed significantly more sonographically diagnosed destructive changes in the TMJ than did patients with <5 affected joints. There was no significance between the number of affected peripheral joints and disc dislocation in the closed-mouth position. In the maximum open-mouth position, there was a significant correlation between the number of affected peripheral joints and disc dislocation. Patients with a JIA duration >23 months had a significantly higher rate of disc dislocation and destructive changes. Patients with a JIA duration >60 months had a significantly higher rate of destructive changes of the TMJ than patients with a disease duration <60 months, but no statistical significance was found concerning disc dislocation. CONCLUSION: The significant correlation between pathologic sonographic findings, duration of JIA, and the number of affected peripheral joints make the technique interesting for use as a diagnostic screening method.

Bone mass development and bone metabolism in juvenile idiopathic arthritis: treatment with growth hormone for 4 years.

OBJECTIVE: To study the acquisition of bone mass and changes in bone mineral density (BMD) related to age, bone age, pubertal status, and growth hormone (GH) therapy in 11 children with juvenile idiopathic arthritis (JIA) longitudinally over 4 years, in comparison to healthy children. METHODS: Bone mineral content (BMC), BMD, and vertebral area were measured by dual energy x-ray absorptiometry. Since BMC and BMD increase with size, BMD was converted to volumetric BMD (vBMD) after adjustment for vertebral size. RESULTS: At inclusion all patients (7 female, 4 male, mean age 10.3 +/- 2.1 yrs) had low BMD, with a mean z-score for area BMD (aBMD) of -2.04 +/- 0.8 SD. After adjustment for size, vBMD was 0.198 g/cm3, and after 4 years of GH treatment it increased significantly to 0.232 g/cm3 (p < 0.03), expressed as SD scores that increased from -2.97 +/- 0.81 SD to -2.83 +/- 0.67 SD. In relation to bone age, vBMD SD increased from -2.53 +/- 0.85 to -2.41 +/- 0.79. Compared to pretreatment values, bone formation and resorption markers increased significantly during treatment. CONCLUSION: Our results reflect an increase in bone turnover under GH therapy in these patients. Despite biochemical changes there was a stabilization of vBMD for age and bone age, with a percentage increase comparable to healthy children. Longterm GH treatment will be necessary to evaluate a potential positive effect of GH on bone density and metabolism in patients with JIA.