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Objective To perform a retrospective study comparing clinical and laboratory aspects of patients with acute rheumatic fever (ARF) and patients with post-streptococcal reactive arthritis (PSRA), to discern whether these are 2 separate entities or varying clinical manifestations of the same disease. Study design We located the records of 68 patients with ARF and 159 patients with PSRA, whose diseases were diagnosed with standardized criteria and treated by 8 pediatric rheumatologists in 7 medical centers, using the Israeli internet-based pediatric rheumatology registry. The medical records of these patients were reviewed for demographic, clinical, and laboratory variables, and the data were compared and analyzed with univariate, multivariate, and discriminatory analysis. Results Four variables were found to differ significantly between ARF and PSRA and serve also as predictors: sedimentation rate, C-reactive protein, duration of joint symptoms after starting anti-inflammatory treatment, and relapse of joint symptoms after cessation of treatment. A discriminative equation was derived that enabled us to correctly classify >80% of the patients. Conclusion On the basis of simple clinical and laboratory variables, we were able to differentiate ARF from PSRA and correctly classify >80% of the patients. It appears that ARF and PSRA are distinct entities.

The aim of this study was to describe the clinical manifestations and outcomes of a national cohort of childhood systemic lupus erythematosus (cSLE). All cases of cSLE registered in the Israeli national registry of children with rheumatic diseases between 1987–2003 were examined for disease activity and damage by the SLE disease activity index (SLEDAI) and SLE collaborating clinics/American College of Rheumatology (SLICC/ACR) damage index. Demographic, clinical, laboratory and treatment factors were analysed for their effect on the outcome. One-hundred and two patients were identified, 81% females, with a mean age at diagnosis of 13.3 ± 2.6 years. The mean SLEDAI score was 17.2 ± 9.0 (range 2–60). Fifty four patients were followed for at least five years. The mean SLEDAI decreased to 7.6 ± 6.3 (0–29) and the mean SLICC/ACR damage index was 0.7 ± 1.6 (0–8). Five patients developed chronic renal failure. No patients died. No factors were found to be significantly associated with the outcome except the initial SLEDAI score. The five-year outcome of our national cSLE cohort was good; with relatively low activity and minimal damage in most patients. The initial SLEDAI predicted the development of late damage.

We performed a prospective, randomized, open-label equivalence study comparing the use of naproxen to aspirin in 33 patients with rheumatic fever. The mean time until resolution of arthritis was 2.9+/-2.9 days in both groups. Liver enzyme elevations were more frequent in the aspirin group (P=.002). We conclude that naproxen is as effective, is easier to use, and is safer than aspirin in the treatment of the arthritis of rheumatic fever.

Objectives Juvenile idiopathic arthritis (JIA) is a chronic inflammatory joint disease affecting children. Even if remission is successfully induced, about half of the patients experience a relapse after stopping anti-inflammatory therapy. The present study investigated whether patients with JIA at risk of relapse can be identified by biomarkers even if clinical signs of disease activity are absent. Methods Patients fulfilling the criteria of inactive disease on medication were included at the time when all medication was withdrawn. The phagocyte activation markers S100A12 and myeloid-related proteins 8/14 (MRP8/14) were compared as well as the acute phase reactant high-sensitivity C reactive protein (hsCRP) as predictive biomarkers for the risk of a flare within a time frame of 6 months. Results 35 of 188 enrolled patients experienced a flare within 6 months. Clinical or standard laboratory parameters could not differentiate between patients at risk of relapse and those not at risk. S100A12 and MRP8/14 levels were significantly higher in patients who subsequently developed flares than in patients with stable remission. The best single biomarker for the prediction of flare was S100A12 (HR 2.81). The predictive performance may be improved if a combination with hsCRP is used. Conclusions Subclinical disease activity may result in unstable remission (ie, a status of clinical but not immunological remission). Biomarkers such as S100A12 and MRP8/14 inform about the activation status of innate immunity at the molecular level and thereby identify patients with unstable remission and an increased risk of relapse.

To obtain longitudinal data on growth/puberty in a large-scale, multi-national prospective cohort of juvenile systemic lupus erythematosus (SLE).Data from 331/557 (59.4%) patients ≤18 years old with juvenile SLE in active phase, with anthropometric data available at four follow-up visits, were studied.There was a significant reduction in parent-adjusted height z score with time in females and males (p0.0001), with a significant gender difference (p0.0001) and with male height being most affected. Median body mass index z score peaked at 6 months and was still significantly above baseline after 26 months (p0.01), with no gender difference. Standardised height reduction was inversely related to age at onset. Females with onset age12 years had a median parent-adjusted height z score of -0.87 with no catch-up growth. At the end of the study, growth failure was seen in 14.7% of the females and 24.5% of the males. Height deflection (less than -0.25/year) was found in 20.7% of the females and 45.5% of the males. Delayed pubertal onset was seen in 15.3% and 24% of the females and males, respectively, and delayed/absent menarche was seen in 21.9%, while 36.1% of the females and 44% of the males had some degree of delayed pubertal development. Growth failure baseline determinants were previous growth failure (OR: 56.6), age at first visit ≤13.4 years (OR: 4.2) and cumulative steroid dose426 mg/kg (OR: 3.6).The children at risk of having a negative effect on height and pubertal development are prepubertal and peripubertal children treated with400 mg/kg cumulative dose of corticosteroids.

Background Intra-articular corticosteroid injection in juvenile idiopathic arthritis (JIA) is often associated with anxiety and pain. Recent reports advocate the use of nitrous oxide (NO), a volatile gas with analgesic, anxiolytic and sedative properties. Objective To prospectively evaluate the effectiveness and safety of NO analgesia for intra-articular corticosteroid injection in JIA, and to assess patients and staff satisfaction with the treatment. Methods NO was administered to JIA patients scheduled for joint injection. The patient, parent, physician and nurse completed visual-analog scores (VAS) (0–10) for pain, and a 5-point satisfaction scale. Change in heart rate (HR) during the procedure was recorded in order to examine physiologic response to pain and stress. Patient's behavior and adverse reactions were recorded. Results 54 procedures (72 joints) were performed, 41 females, 13 males; 39 Jewish, 13 Arab; mean age was 12.2 ± 4.7 year. The median VAS pain score for patients, parents, physicians and nurses was 3. The HR increased ≥ 15% in 10 patients. They had higher VAS scores as evaluated by the staff. The median satisfaction level of the parents and staff was 3.0 and 5.0 respectively. Adverse reactions were mild. Conclusion NO provides effective and safe sedation for JIA children undergoing intra-articular injections.

Purpose: We sought to describe cases of optic neuritis associated with etanercept therapy. Methods: A retrospective chart review was undertaken on all patients that developed uveitis or optic neuritis associated with etanercept therapy between January 2003 and January 2005 in 2 medical centers: Assaf Harofeh Medical Center, and Kaplan Medical Center, Israel. Results: Four patients (3 girls, 1 boy) treated with etanercept for juvenile idiopathic arthritis (JIA) are presented. The 3 girls had oligoarticular-onset JRA. The boy had HLA-B27-positive juvenile spondyloarthropathy and bilateral uveitis. After a mean follow-up of 10 months (range, 2.5-18 months) all 4 patients had reduced visual acuity due to optic neuritis, which was accompanied by vitreitis in 2 eyes. In 3 patients, the discontinuation of etanercept, together with steroid treatment, resulted in resolution of the inflammation. The fourth patient elected to continue etanercept treatment and experienced no further deterioration in visual acuity. Conclusion: Optic neuritis is a potentially sight-threatening complication of etanercept therapy. Patients with JRA who are candidates for therapy should be examined by an ophthalmologist before starting treatment and then regularly thereafter. Ophthalmologists and rheumatologists should be aware of this hazard and be cautious when using etanercept in this patient population.

SummaryIn Israel, vaccinations are the overall responsibility of the government. We were the first in Israel to give the Hib (Haemophilus influenza type b) vaccine to the population, through independent means, without government control. The aim of the study was to follow longitudinally the specific group of children vaccinated in our ambulatory clinic. In this study, 1,497 children between 2 and 52 [mean (SD) 13 (9)] months of age at the time of first vaccination were vaccinated with Hib vaccine. Over the next 7 years, they were followed up by repeated phone calls when parents were asked about hospitalisation and any serious infectious diseases. Of the 1,497, 1,444 were followed during the years 1992 to 1999 and 36 were hospitalised during this time. All blood and cerebrospinal fluid cultures were negative. No proven case of Hib infection could be demonstrated. Despite the small sample size, this study justifies the continued use of the vaccine along with maintaining surveillance for Hib infection.

Hereditary sensory neuropathy type IV is an autosomal-recessive disorder characterized by congenital insensitivity to pain and anhidrosis and resulting in recurrent hyperpyrexia, self-mutilation, recurrent infections, chronic osteomyelitis, bone and joint deformities, and limb amputations. Described is a child with signs as well as skin and nerve biopsy results compatible with this disease, emphasizing the importance of early diagnosis and appropriate medical and educational care to prevent complications.

Idiopathic thrombocytopenic purpura (ITP) in childhood is a benign disease, as only 10% to 20% of (he patients have a chronic course. A retrospective study of 57 ITP patients ranging in age from four months to two years revealed that 30% of them proceeded to chronicity. Unlike ITP in the general pediatric population, chronic infantile ITP was characterized by male predominance, a high frequency of preceding viral infections, and lack of responsiveness to any of the known modalities of treatment.