Standardized reporting and quantification of whole-body MRI findings in children with chronic non-bacterial osteomyelitis treated with pamidronate.

OBJECTIVES: The objectives were to assess changes in radiological disease activity in children with chronic non-bacterial osteomyelitis (CNO) receiving pamidronate therapy and to test a modified radiological index for non-bacterial osteitis (mRINBO) in CNO. mRINBO was used for standardized reporting and quantification of whole-body MRI (WBMRI) findings resulting in an individual summary patient score. METHODS: WBMRI was retrospectively assessed in 18 children with CNO at baseline and after receiving pamidronate therapy for one year. Parameters of interest were: number and anatomic site of radiologically active bone lesions (RAL), size of RAL, extramedullary affection, spinal involvement and changes in mRINBO, which includes both the number and maximal size of RAL (RALmax) in addition to extramedullary and chronic changes. RESULTS: At the time of diagnosis, the mean age of the children was 9.8 (sd, 8.7-10.9) years and 11/18 were females. The number of RALs per patient decreased from median [interquartile range] 4.5 [3-8] to 3 [2-5] RALs per patient (p = 0.02) and extramedullary inflammatory changes regressed. Sixty-one percent of all RALs occurring at baseline resolved and three children became without active inflammatory lesions by WBMRI. The median size of RALs did not change when taking new lesions occurring in 7/18 children into account, but RALmax decreased significantly from 39 [29-45] mm at baseline to 28 [20-40] mm (p < 0.01) at year-one with a concomitant decrease of mRINBO from a median of 5 [4-7] to 4 [3-5] (p = 0.05). CONCLUSIONS: Pamidronate therapy resulted in a decrease of mRINBO from baseline to year one. mRINBO may be a potential scoring method to quantify changes in radiological disease activity in children with CNO. However, further studies are needed to test feasibility and validity of mRINBO.

Pamidronate in chronic non-bacterial osteomyelitis: a randomized, double-blinded, placebo-controlled pilot trial.

OBJECTIVE: This is the first randomized double-blinded, placebo-controlled pilot trial to investigate the efficacy of pamidronate in reducing radiological and clinical disease activity in chronic non-bacterial osteomyelitis (CNO). METHOD: Patients received pamidronate or placebo at baseline and weeks 12 and 24. Whole-body magnetic resonance imaging was performed at baseline and weeks 12 and 36, and computed tomography of the anterior chest wall (ACW) at baseline and week 36. Radiological disease activity was systematically scored in the ACW and spine. Patient-reported outcomes [visual analogue scale (VAS) pain, VAS global health, Health Assessment Questionnaire (HAQ), EuroQol-5 Dimensions (EQ-5D), and 36-item Short-Form Health Survey (SF-36)] and biomarkers of bone turnover and inflammation were assessed at baseline and weeks 1, 4, 12, 24, and 36. Data are expressed as median [interquartile range]. RESULTS: Fourteen patients were randomized and 12 were analysed. From baseline to week 36, the radiological disease activity score in the ACW decreased from 5 [4-7] to 2.5 [1-3] in the pamidronate group, but did not change in the placebo group (p = 0.04). From baseline to week 36, VAS pain and VAS global health tended to decrease more in the pamidronate than in the placebo group (p = 0.11, p = 0.08). Physical functioning (HAQ) and health-related quality of life (EQ-5D, SF-36) did not change. Biomarkers of bone turnover decreased only in the pamidronate group (p ≤ 0.02). CONCLUSION: Pamidronate may improve radiological and clinical disease activity in CNO. Methods to score radiological disease activity in adult CNO were suggested. Clinical Trials: NCT02594878.

Defining consensus opinion to develop randomised controlled trials in rare diseases using Bayesian design: An example of a proposed trial of adalimumab versus pamidronate for children with CNO/CRMO.

INTRODUCTION: Chronic nonbacterial osteomyelitis (CNO) is a rare autoinflammatory bone disorder primarily affecting children and adolescents. It can lead to chronic pain, bony deformities and fractures. The pathophysiology of CNO is incompletely understood. Scientific evidence suggests dysregulated expression of pro- and anti-inflammatory cytokines to be centrally involved. Currently, treatment is largely based on retrospective observational studies and expert opinion. Treatment usually includes nonsteroidal anti-inflammatory drugs and/or glucocorticoids, followed by a range of drugs in unresponsive cases. While randomised clinical trials are lacking, retrospective and prospective non-controlled studies suggest effectiveness of TNF inhibitors and bisphosphonates. The objective of the Bayesian consensus meeting was to quantify prior expert opinion. METHODS: Twelve international CNO experts were randomly chosen to be invited to a Bayesian prior elicitation meeting. RESULTS: Results showed that a typical new patient treated with pamidronate would have an 84% chance of improvement in their pain score relative to baseline at 26 weeks and an 83% chance on adalimumab. Experts thought there was a 50% chance that a new typical patient would record a pain score of 28mm (pamidronate) to 30mm (adalimumab) or better at 26 weeks. There was a modest trend in prior opinion to indicate an advantage of pamidronate vs adalimumab, with a 68% prior chance that pamidronate is superior to adalimumab by some margin. However, it is clear that there is considerable uncertainty about the precise relative merits of the two treatments. CONCLUSIONS: The rarity of CNO leads to challenges in conducting randomised controlled trials with sufficient power to provide a definitive outcome. We address this using a Bayesian design, and here describe the process and outcome of the elicitation exercise to establish expert prior opinion. This opinion will be tested in the planned prospective CNO study. The process for establishing expert consensus opinion in CNO will be helpful for developing studies in other rare paediatric diseases.

Clinical Outcome and Long-term Remission in JIA.

PURPOSE OF REVIEW: This review assesses the long-term remission and predictors of clinical outcome in patients with juvenile idiopathic arthritis (JIA). A comprehensive literature search was performed including articles published between January 1, 2004 and February 28, 2017. Studies, with a minimum follow-up of 24 months, were selected independently by two reviewers based on in- and exclusion criteria. The objective outcome was inactive disease/clinical remission as defined by the Wallace criteria at last follow-up. RECENT FINDINGS: The probability of achieving inactive disease and/or clinical remission is dependent on the JIA subcategories studied in the different articles. Overall, a significant proportion of JIA patients still showed signs of active disease at last follow-up. Some studies include patient populations followed for 15 years or more and these patients were exposed to different treatment protocols at disease presentation than patients diagnosed in the biologic era. Although the severity of the morbidity and associated mortality risk has decreased over time, a significant proportion of the current JIA patients still do not reach an inactive disease status within a 2-year follow-up window. Studying the long-term outcome of patients with JIA remains challenging due to the heterogeneity of the study designs and study populations. Although improvement has been shown in the biologic era, we still need to enhance the number of patients with inactive disease within the first 2 years after diagnosis.

Remission rate is not dependent on the presence of antinuclear antibodies in juvenile idiopathic arthritis.

Recently, it has been hypothesized that the subcategories of the ILAR classification of juvenile idiopathic arthritis (JIA) are not homogeneous, and that the presence of antinuclear antibodies (ANA) should lead to a separate entity. Therefore, the aim of this study was to evaluate ANA positivity as a predictor of achieving remission. A retrospective single-center cohort study including all JIA patients diagnosed between January 2000 and May 2014. A minimum follow-up of 1 year was required plus the ANA status. ANA positivity was defined as at least two positive results with a titer ≥1:160. Demographic and clinical features were collected. Remission at last follow-up was defined by the Wallace criteria. A total of 625 patients met the inclusion criteria and 230 (37%) were found ANA positive. Analysis showed no difference in remission rate between ANA-positive and ANA-negative patients. Additionally, joint count at diagnosis and at last follow-up were comparable in both groups. ANA positivity was correlated to a female predominance and young age at diagnosis (p < 0.001). Remission rates are not different in ANA-positive patients than in ANA-negative patients. This does not support the hypothesis to possibly divide JIA patients based on their ANA status.

Accelerometer-assessed daily physical activity in relation to pain cognition in juvenile idiopathic arthritis.

OBJECTIVES: Pain has been known to predict low physical activity (PA) in juvenile idiopathic arthritis (JIA) and high levels of pain are related to maladaptive coping rather than disease severity. Objectively monitored PA in JIA has recently been shown not to be related to pain intensity, emphasizing the need to explore pain more deeply. The aim of this study was to examine accelerometer-assessed PA in relation to pain cognition in children with JIA. METHOD: Data gathered included disease activity, functional ability, and pain cognition. PA was monitored with a GT1M Actigraph accelerometer. RESULTS: Sixty-one patients were included. Disease activity, functional impairment, and pain intensity scores were relatively low. Accelerometry was correlated positively to the specific belief of having control of pain ('Control') but negatively to disease activity. There was no correlation with functional ability, pain intensity, coping strategies, or other pain beliefs. When isolated, disease activity (measured by the 27-joint count Juvenile Arthritis Disease Activity Score, JADAS-27) contributed significantly to the variance in accelerometry, while 'Control' could not significantly explain a unique part of the variance. CONCLUSIONS: Objectively measured PA was negatively correlated to disease activity but not to pain intensity. The only pain cognition measurement to reach higher levels of PA was to be in control of pain.

Normative values for mandibular mobility in Scandinavian individuals 4-17 years of age.

Assessment of mandibular mobility is an important part of the clinical oro-facial examination of paediatric and adolescent patients. The aims of the present cross-sectional study were to establish age-related normative values for mandibular mobility in a Scandinavian paediatric and adolescent cohort and to assess the validity of universal cut-off values for lower 'normal' mandibular ranges of motion. A total of 1114 Danish individuals between 4-17 years of age were included. Maximal mouth opening capacity and laterotrusion capacity were assessed, in each individual, according to a standardised measurement protocol. The mean maximal mouth opening capacity gradually increased from 38 mm (SD 6·1 mm) at age 4 to 54·5 mm (SD 6·8 mm) at age 17. No inter-gender difference in maximal mouth opening capacity was observed (P > 0·15). The mean maximal laterotrusion capacity gradually increased from 7·4 mm (SD. 1·1 mm) at age four to 10·1 mm (SD 1·9 mm) at age 17. A statistical significant inter-gender difference of 0·8 mm (SD 0·4 mm) was observed in relation to the total laterotrusion capacity; however, the clinical relevance of this significant difference is questionable. Normative values of mandibular function was established in individuals 4-17 years of age. Our findings oppose the use of a single universal cut-off value for 'normal' range of motion in paediatric and adolescent patients. Instead, we recommend to use the age-related normative values of mandibular range of motion as basis for the assessment of the development of oro-facial function.

Accelerometry-based monitoring of daily physical activity in children with juvenile idiopathic arthritis.

OBJECTIVES: Juvenile idiopathic arthritis (JIA) may cause functional impairment, reduced participation in physical activity (PA) and, over time, physical deconditioning. The aim of this study was to objectively monitor daily free-living PA in 10-16-year-old children with JIA using accelerometry with regard to disease activity and physical variables and to compare the data with those from healthy age- and gender-matched controls. METHOD: Patients underwent an evaluation of disease activity, functional ability, physical capacity, and pain. Accelerometer monitoring was assessed using the GT1M ActiGraph. Normative data from two major studies on PA in Danish schoolchildren were used for comparison. RESULTS: Data of accelerometry were available for 61 JIA patients and 2055 healthy controls. Of the JIA patients, 57% showed below-average values of maximal physical capacity (fitness level). JIA patients showed low disease activity and pain and were physically well functioning. Accelerometer counts were lower in JIA patients than in controls. Accelerometer measurements were negatively correlated with disease activity, erythrocyte sedimentation rate (ESR), and number of joints with swelling and/or limited range of motion (ROM). No correlation was found between PA and pain scores, functional ability, and hypermobility. Patients with involvement of ankles or hips demonstrated significantly lower levels of PA. CONCLUSIONS: Children with JIA are less physically active and have lower physical capacity and fitness than their age- and gender-matched healthy peers despite good disease control. The involvement of hips or ankles is associated with lower PA.

Cytokines in healthy temporomandibular joint synovial fluid.

Analysis of temporomandibular joint (TMJ) synovial fluid may elucidate the aetiology of temporomandibular disorders and arthritic conditions, as well as the inflammatory mechanisms involved. Knowledge about healthy synovial fluid is necessary to understand TMJ pathologies. We aimed to quantify the proinflammatory cytokines interleukin (IL)-1ß, IL-2, IL-6 and tumour necrosis factor (TNF), and the anti-inflammatory cytokines IL-10 and interferon (IFN)-γ in healthy TMJ synovial fluid to serve as reference values for future studies on TMJ pathologies. Twenty healthy, young adult volunteers without temporomandibular dysfunction were included. Bilateral synovial fluid samples were obtained using the push-pull technique with hydroxocobalamin described by Alstergren in 1999. Cytokines were quantified with Luminex multiplex assays and compared using nonparametric statistical analysis. No serious adverse effects were reported. Of 40 possible samples, 14 fulfilled the strict sampling criteria and were included in the analysis. Cytokine values (reported as medians with interquartile ranges) were as follows: TNF, 23 (13-37) pg mL(-1) ; IL-2, 1·8 (0-22) pg mL(-1) ; and INF-γ, 10 (0-47) pg mL(-1) . IL-1ß, IL-6 and IL-10 were almost undetectable. In addition, TNF and INF-γ cytokine levels correlated. We demonstrated that TNF was consistently detected and IFN-γ and IL-2 sporadically detected in the TMJ synovial fluid of healthy individuals using the hydroxocobalamin method and a multiplex assay. The cytokines IL-10, IL-1ß and IL-6 were barely detectable in this sample of healthy TMJs.

Management of temporomandibular joint arthritis-related orofacial symptoms in juvenile idiopathic arthritis by the use of a stabilization splint.

OBJECTIVES: Temporomandibular joint (TMJ) inflammation in patients with juvenile idiopathic arthritis (JIA) may interfere with optimal joint and muscle function. Orofacial symptoms are common findings in relation to TMJ arthritis. Previous research on management of TMJ arthritis-related orofacial symptoms in patients with JIA has focused on pharmacological treatment modalities without involving physical pain management strategies. The aim of this study was to evaluate changes in orofacial pain and TMJ function after stabilization splint treatment. METHOD: Twenty-eight consecutive patients with JIA and arthritis-related orofacial symptoms (mean age 15.5 years, range 8.2-25 years) were included in this prospective observational study. All patients received stabilization splint treatment. A comparable group of 110 healthy children served as controls for the assessments of TMJ mobility. RESULTS: After splint treatment for 8 weeks, a significant reduction in orofacial pain frequency and intensity was reported, and significant improvement in TMJ function was observed. However, TMJ mobility at follow-up remained significantly reduced when compared to the control group. CONCLUSIONS: The stabilization splint is a safe, reversible, low-cost treatment, and familiar to most dental practitioners. Based on our findings, we propose the implementation of stabilization splint therapy for the treatment of JIA patients with TMJ arthritis-related symptoms.

The relevance of using the Childhood Health Assessment Questionnaire (CHAQ) in revised versions for the assessment of juvenile idiopathic arthritis.

OBJECTIVES: The Childhood Health Assessment Questionnaire (CHAQ) is widely used to measure functional impairment in juvenile idiopathic arthritis (JIA). However, the original version (CHAQ30) has reduced overall validity in terms of an increasing ceiling effect and decreasing discriminative ability because of considerable improvements in functional ability of children with JIA. The aim of this study was to validate a revised CHAQ including eight new physically demanding questions (CHAQ38) with two categorical response and scoring models (CAT1 and CAT2) in Danish children with JIA and healthy controls of a comparable age and gender. The CHAQ versions with both response models were validated in relation to distribution of data, evaluation of the ceiling effect, sensitivity, and discriminative ability. METHOD: Four different version of the CHAQ were completed by 68 patients and 118 controls aged 10-16 years. Demographic data in both groups and disease-specific data among patients were obtained. Statistical analysis of all CHAQ versions was performed to evaluate score distribution and to test the ability to distinguish between patients and controls. RESULTS: The use of the CHAQ38 broadened the range of scores and reduced the number of scores at zero. The ceiling effect, which was high in all versions, was best reduced using the CAT2 response model. Adding the new questions increased the sensitivity to discriminate between physically well-functioning JIA patients and healthy controls in either of the CAT models used. Overall, the CHAQ38 and to some extent the CAT2 had the best outcomes. CONCLUSIONS: The discriminative validity increased with the CHAQ38, though still not optimally.

Smallest detectable differences in clinical functional temporomandibular joint examination variables in juvenile idiopathic arthritis.

OBJECTIVE: Temporomandibular joint (TMJ) arthritis in juvenile patients may interfere with optimal joint function and mouth opening patterns. Clinical assessment of maximal mouth opening capacity, laterotrusion and protrusion is critical to TMJ arthritis diagnosis, treatment choice and evaluation of a therapeutic intervention. The aim of the study was to determine the smallest minimal threshold at which differences in maximal mouth opening capacity, laterotrusion, and protrusion between two consecutive observations can be determined. SETTING AND SAMPLE POPULATION: Department of Orthodontics, University of Aarhus, Denmark. Forty-two consecutive patients with juvenile idiopathic arthritis. MATERIAL AND METHODS: Two experienced dentists used a calibrated metallic ruler to measure maximal mouth opening capacity, laterotrusion, and protrusion. Each measurement was carried out thrice by each observer. Intra- and inter-observer variation and the smallest detectable difference were calculated for each variable. RESULTS: The smallest detectable differences were as follows: maximal mouth opening capacity 4.9 mm, laterotrusion 2.4 mm, and protrusion 2.8 mm (one observer and one measurement). These differences declined when measurements were repeated; maximal mouth opening capacity 3.3 mm, laterotrusion 1.4 mm, and protrusion 1.8 mm (two observers with three measurements each). We found no support for a relationship between measurement variation and patient age, measurement variation and TMJ pain, or between measurement variation and previous/current TMJ arthritis. CONCLUSION: The importance of the implementation of a standardized measurement protocol is emphasized including repeated measurements to reduce the smallest detectable difference.

Functional changes following distraction osteogenesis treatment of asymmetric mandibular growth deviation in unilateral juvenile idiopathic arthritis: a prospective study with long-term follow-up.

In juvenile idiopathic arthritis (JIA), temporomandibular joint involvement is a frequent complication leading to deficient mandibular growth. Occurring unilaterally this will give rise to mandibular and maxillary asymmetry that will affect the soft tissue and the muscles and result in complex dentofacial anomaly. In the case of severe dentofacial malformation, orthognathic surgery is the only treatment option. Vertical osseodistraction of the mandibular ramus has been suggested as a means of rectifying the mandibular growth deviation and soft-tissue problems. Whether such treatment introduces dysfunctional side effects of the temporomandibular joint and muscles has been debated and concern has been raised that treatment impairs the patient's mouth opening capacity and mandibular movement. The present study prospectively evaluated 23 patients with JIA and mandibular asymmetry caused by unilateral temporomandibular joint arthritis. The authors found a clinical effect on the asymmetry with only minor subjective complaints and limited objective changes in functional parameters.

Validity and predictive ability of the juvenile arthritis disease activity score based on CRP versus ESR in a Nordic population-based setting.

OBJECTIVE: To compare the juvenile arthritis disease activity score (JADAS) based on C reactive protein (CRP) (JADAS-CRP) with JADAS based on erythrocyte sedimentation rate (ESR) (JADAS-ESR) and to validate JADAS in a population-based setting. METHODS: The CRP and ESR values and the corresponding JADAS scores (JADAS10/27/71) were compared in a longitudinal cohort study of 389 children newly diagnosed with juvenile idiopathic arthritis (JIA) in the Nordic JIA study. The construct validity and the discriminative and predictive ability of JADAS were assessed during a median disease course of 8 years by comparing JADAS with other measures of disease activity and outcome. RESULTS: At the first study visit the correlation between JADAS27-CRP and JADAS27-ESR was r=0.99 whereas the correlation between CRP and ESR was r=0.57. Children with higher JADAS scores had an increased risk of concomitant pain, physical disability and use of disease-modifying antirheumatic drugs (DMARDs). A higher JADAS score at the first study visit also significantly predicted physical disability, damage and no remission off medication at the final study visit, and also use of DMARDs during the disease course. Sensitivity to change, demonstrated as change in JADAS score compared with the American College of Rheumatology paediatric measures of improvement criteria, mostly showed excellent classification ability. CONCLUSION: The JADAS-CRP and JADAS-ESR correlate closely, show similar test characteristics and are feasible and valid tools for assessing disease activity in JIA.

Long-term outcome in patients with juvenile dermatomyositis: a cross-sectional follow-up study.

OBJECTIVE: To evaluate a group of 53 patients with juvenile dermatomyositis (JDM), on average 13.9 years after disease onset, in order to describe the long-term disease outcome and to identify disease-related parameters associated with poor disease outcome. METHODS: Baseline information at disease onset was obtained from medical records. Disease status at follow-up was evaluated by physical examination. The Myositis Damage Index (MDI) and the Myositis Disease Damage by Visual Analogue Scale (MYODAM-VAS) were used to describe disease damage. RESULTS: Disease damage was seen in 60.4% of patients. The most common damage was cutaneous scarring (39.6%) and muscle dysfunction (34%). Calcinosis was found in 20%, lipodystrophy in 13%, and severe damage affecting more than two organ systems in 24.5% of patients. A disease duration > 4 years increased the risk of damage based on: MDI score [adjusted odds ratio (AOR) 8.3, 95% confidence interval (CI) 1.7-41.7], MYODAM-VAS score (AOR 26.2, 95% CI 3.1-223.7), and number of affected organs (AOR 16.3, 95% CI 1.1-232.4). Disease onset age ≥ 7.4 years increased the risk of more than two affected organs (AOR 15.8, 95% CI 1.9-129.4). Disease duration ≥ 4 years increased the risk of calcinosis (OR 4.8, 95% CI 1.1-20.9) and continuous muscle dysfunction (OR 4.2, 95% CI 1.1-17.3). CONCLUSION: In a long-term follow-up study, 60% of JDM patients had disease damage at a mean of 14 years after disease onset. Longer disease duration was the most important predictor of damage, calcinosis, and impaired muscle function, and higher age at disease onset predicted more organs involved at follow-up.

Ultrasound and MRI measurements of joint cartilage in healthy children: a validation study.

PURPOSE: In juvenile idiopathic arthritis (JIA), proliferative changes in the synovium and synovial fluid accumulation are pathological findings responsible for damage to the cartilaginous tissue and periarticular bone, which are late radiographic findings in conventional radiography. Early detection of these joint changes would allow the clinicians to initiate relevant therapies as is essential for the long-term outcome of JIA. Ultrasonography (US) has shown great potential for this purpose but validation in a pediatric setting is needed. The objective of this study was to validate US measurements of cartilage thickness in target joints in healthy children by comparing them with MRI. MATERIALS AND METHODS: Twenty-five healthy Caucasian children (17 boys/ 8 girls), mean age 11.33 years, were examined with MRI (1.5 T, fat-suppressed T 1-weighted 3D sequences) and US (real-time Hitachi EUB 6500 CFM, B-mode 6 - 14 MHz linear transducer) in the right knee, ankle, wrist, metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints. US was obtained according to the EULAR standard scans. RESULTS: All differences in cartilage thickness measurements between MRI and US were less than 0.5 millimeters. The coefficient of variation (CV) was found to be good (16 %) except for in the case of the wrist joint (20 %). CONCLUSION: We found a good level of agreement and no significant systematic joint size-related differences in cartilage thickness measurements between MRI and US. US appears to be a feasible method for evaluating cartilage thickness in JIA target joints, especially when age and sex-related references are defined.

Clinical features and outcome in a Danish cohort of juvenile dermatomyositis patients.

OBJECTIVES: To assess disease characteristics and outcome in Danish juvenile dermatomyositis (JDM) patients (1977-2007). METHODS: Medical record review of hospital records identified from the National Patient Register. RESULTS: Fifty-seven JDM patients were identified. Follow-up time was 7 years (range 0.06-30). Female:male ratio was 2.5:1. Mean age at disease onset was 7 years (SD±3.7), range 1.5-16.0 years. Diagnostic delay was 0.7 years (SD±1.6), range 0.04-9 years. Mean disease duration was 3.7 years (SD±3.5), range 0.7-9 years. Thirty-nine patients (70%) were in full remission. Three patients (5%) were deceased. Disease/treatment-induced damage was present in 35 (61%) patients. Decreased pulmonary function occurred early in the disease course (median 10 months), osteoporosis and calcinosis occurred later (median 18 and 22 months). Four patients developed persistent damage within the first 6 months, four developed calcinosis within the first year. Shorter disease duration was associated with less damage (p=0.004). In a multivariate assessment analysis age >10 years at disease onset was associated with more damage (p<0.01), OR 10.96 (CI 1.6-73.6), and disease duration >4 years was associated with calcinosis (p=0.01) OR 23.2 (CI 2.6-206.2). CONCLUSIONS: We present a nationwide retrospective study of Danish JDM patients from 1977-2007. Although 70% were in remission, 61% of the patients had clinical signs of damage. Only a few patients developed damage within the first year of the disease. Longer disease duration and higher age at disease onset was correlated with more disease damage.

Condylar lesions in relation to mandibular growth in untreated and intra-articular corticosteroid-treated experimental temporomandibular joint arthritis.

OBJECTIVES: To evaluate condylar lesions in relation to mandibular growth in experimental temporomandibular joint (TMJ) arthritis and to assess the outcome of treating this condition with repeated intra-articular corticosteroid injections (IACIs). METHODS: Forty-two 10-week-old rabbits were randomly divided into four groups. Seven animals served as controls. Experimental TMJ arthritis was induced in five animals which received intra-articular TMJ saline injections. Fifteen animals had TMJ arthritis induced and were left untreated and 15 animals had TMJ arthritis induced and were treated with IACIs one week after each TMJ antigen-challenge procedure. Inter-group growth differences were evaluated from head computerised tomography scans taken at the time of arthritis induction and 12 weeks later. The variables assessed were: progression of condylar lesions (erosions/flattening/osteophytes), mandibular bone volume changes, condylar and sagittal ramus growth. RESULTS: No inter-group differences in the progression of condylar lesions were observed despite reduced mandibular growth in all three experimental groups. The most pronounced unfavourable mandibular growth alterations were observed in the corticosteroid-treated arthritis animals. CONCLUSIONS: No evidence was found in support of a relation between reduced mandibular growth and condylar lesions. We propose that: 1) condylar lesions are not the only causative factor of reduced mandibular growth in experimental TMJ arthritis, and 2) repeated IACIs have a very unfavourable impact on mandibular growth in experimental TMJ arthritis - treatment is more detrimental to mandibular growth than the TMJ arthritis itself.

Prevention of mother-to-child transmission of HIV in Denmark, 1994-2008.

OBJECTIVES: The aim of this study was to describe trends in the management of pregnancies in HIV-infected women and their outcomes over a 14-year period in Denmark on a national basis. METHODS: The study was a retrospective cohort study of all HIV-infected women in Denmark giving birth to one or more children between 1 June 1994 and 30 June 2008. RESULTS: We identified 210 HIV-infected women with 255 pregnancies, ranging from 7 per year in 1995 to 39 per year in 2006. Thirty per cent of the women were Caucasian and 51% were Black African. Knowledge of HIV status before pregnancy increased from 8% (four of 49) in 1994-1999 to 80% (164 of 206) in 2000-2008. Only 29% (53 of 183) of the women chose to consult an infectious disease specialist when planning pregnancy, while 14% (27 of 199) received assistance with fertility. The proportion of women on antiretroviral therapy (ART) increased from 76% (37 of 49) in 1994-1999 to 98% (201 of 206) in 2000-2008. Vaginal deliveries ranged from 0 in 2003 to 35% of pregnancies in 2007. Mother-to-child transmission (MTCT) of HIV decreased from 10.4% in 1994-1999 to 0.5% in 2000-2008. All women giving birth to an HIV-positive child were diagnosed with HIV during or after delivery and did not receive prophylactic ART. CONCLUSIONS: The annual number of HIV pregnancies increased fivefold during this 14-year period and substantial changes in pregnancy management were seen. No woman treated according to the national guidelines, i.e. ART before week 22, intravenous zidovudine (ZDV) during labour, neonatal ZDV for 4 to 6 weeks and no breastfeeding, transmitted HIV to her child.