Early predictors of longterm outcome in patients with juvenile rheumatoid arthritis: subset-specific correlations

OBJECTIVE: To determine early predictors of longterm outcome in juvenile rheumatoid arthritis (JRA) in a multicenter cohort. METHODS: Patients were selected if they were > or = 8 years of age; the onset of arthritis occurred > or = 5 years before study; and a diagnosis of JRA was made at a participating center. Outcome variables were scores on self-administered Childhood Health Assessment Questionnaires (CHAQ) and active disease duration. Possible explanatory variables assessed included characteristics present at onset, HLA alleles, in particular the rheumatoid arthritis associated shared epitope (RASE), and radiographic indicators of joint damage within 2 years of onset. Data for 393 patients were available. Multivariate analyses were performed for the total group and for each onset subtype. RESULTS: Male sex correlated with worse disability in systemic onset JRA but less disability in RF negative, and a shorter active disease duration in RF positive polyarticular onset JRA. Positive antinuclear antibody correlated with a longer active disease duration in patients with pauciarticular onset JRA. Younger age at onset predicted longer active disease duration in pauciarticular and RF negative polyarticular, and a shorter active disease duration in systemic onset JRA. Residence on a reserve, rather than native North American race, correlated with worse disability. The RASE correlated with less disability in systemic JRA; but no correlation with outcome was evident for patients with rheumatoid factor positive polyarticular JRA. CONCLUSION: Variables predictive of longterm outcome in JRA are specific for each onset subtype. The most important early predictors were age at onset and sex of the patient. Place of residence may have a greater effect on disability than race. RASE may associate with a more favorable outcome in systemic onset disease.     

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

Objective

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.

Methods

Children with rheumatic diseases initiating GC were enrolled in a prospective observational study. Annual spine radiographs were evaluated using the Genant semiquantitative method. Spine areal bone mineral density (aBMD) was measured every 6 months. Clinical features, including cumulative GC dose, back pain, disease and physical activity, calcium and vitamin D intake, and spine aBMD Z scores, were analyzed for association with IVF.

Results

Seven (6%) of 118 children (95% confidence interval 2.9–11.7%) had IVF. Their diagnoses were: juvenile dermatomyositis (n = 2), systemic lupus erythematosus (n = 3), systemic vasculitis (n = 1), and mixed connective tissue disease (n = 1). One child was omitted from the analyses after 4 months because of osteoporosis treatment for symptomatic IVF. Children with IVF received on average 50% more GC than those without (P = 0.030), had a greater increase in body mass index (BMI) at 6 months (P = 0.010), and had greater decrements in spine aBMD Z scores in the first 6 months (P = 0.048). Four (67%) of 6 children with IVF and data to 12 months had spine aBMD Z scores less than ?2.0 at 12 months compared to 16% of children without IVF (P = 0.011).

Conclusion

The incidence of VF 12 months following GC initiation was 6%; most children were asymptomatic. Children with IVF received more GC, had greater increases in BMI, and had greater declines in spine aBMD Z scores in the first 6 months.

     

PSEUDOTUMOUR OF THE ORBIT: BILATERAL METACHRONOUS PRESENTATION

SUMMARY A case is presented of a pseudotumour that developed in the left orbit 4 years after surgery and histological confirmation of a pseudotumour in the right orbit. There was no evidence of any thyroid pathology.     

Angelchik prosthesis with oesophageal adenocarcinoma: our surgical approach

The Angelchik prosthesis is an incomplete doughnut-shaped device composed of silicone elastomer used in the treatment of gastro-oesophageal reflux disease (GORD). It is used to encircle the lower oesophagus at the gastro-oesophageal junction (GOJ). The ease of the operation led to the insertion of over 25,000 such prostheses world-wide. However, a variety of major complications including intractable dysphagia, prosthesis migration and gastric erosion required a quarter of these devices to be removed. Development of adenocarcinoma in patients with Angelchik prosthesis is a rare occurrence. This article describes two patients who developed adenocarcinoma above their prosthesis and whose cardio-oesophagectomy was technically challenging due to the formation of a dense inflammatory capsule around the prosthesis. Our surgical approach to curative oesophageal resection with the Angelchik prosthesis in situ is also discussed.     

Favourable Long-term Results with the End-to-Side Jejunoileal Bypass

Background: Although jejunoileal bypass (JIB) causes longstanding weight loss, it is no longer recommended as a surgical treatment of morbid obesity due to adverse effects. Methods: JIB was performed on 87 morbidly obese subjects with a mean age of 35 years. Complete followup on 95% of the patients included monitoring weight, metabolic parameters and liver biopsies up to 25 years postoperatively. Results: The mean (± sd) Body Mass Index (BMI) was reduced from 41.5 ± 5.8 kg m⁻² preoperative, to 26.7 ± 3.8 kg m⁻² at 2 years and 29.7 ± 3.9 kg m⁻² at 16 years follow-up. More than 60% loss of initial excess weight was achieved by 88% of the patients at four years and by 75% at 16 years follow-up. Reversal of the bypass was performed in 3% of the patients and revisions in 8% of the patients. There was no 30-day hospital mortality but there was one (1%) late bypass-related death. Complications included urinary calculi in 39% of the patients, electrolyte disturbances in 25% and transient liver failure in 5.5%. Liver biopsies taken more than 13 years postoperatively in 44 patients revealed no cirrhosis. All patients were normoglycemic and normolipemic at follow-up. Conclusions: The majority of the patients have an acceptable weight reduction, few serious adverse effects but several beneficial effects after more than 16 years. The JIB deserves a reconsideration as an alternative in obesity surgery.     

IL-2 production in the autologous mixed lymphocyte reaction of patients with juvenile rheumatoid arthritis.

Interleukin 2 (IL-2) production in the CD4/non-T cell autologous mixed lymphocyte reaction (AMLR) was estimated for patients with juvenile rheumatoid arthritis (JRA). A cellular IL-2 assay (CILA) consisting of co-cultures of indicator cells, CTLL-2, added to CD4/non-T cell AMLR cultures was used. As both CD4 and CTLL-2 cell proliferation are IL-2 dependent, results of the CILA assay were taken as a reflection of the total IL-2 in the co-culture. By this assay most patients with JRA had normal IL-2 production. However, five patients with polyarticular disease had both a low AMLR and low levels of IL-2 production. Three of those tested showed little improvement in the AMLR on addition of recombinant IL-2, suggesting decreased responsiveness to IL-2 as well.