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Background: Studies of paediatric patients with membranous lupus nephritis (MLN) have yielded variable results, mostly due to the inclusion of mixed, i.e. proliferative nephritis. The aim of this study was to describe clinical and laboratory findings at the diagnosis of paediatric non-proliferative MLN, report the outcome and identify predictors of remission., Methods: A single-center cohort study of consecutive children diagnosed with non-proliferative MLN was performed. Clinical and laboratory measures and treatment regimens were obtained in prospective standardized assessments. Renal outcome was measured by renal parameters and steroid requirement. Predictors for remission and time to remission were determined., Results: A total of 30 children were identified with a median follow-up time 4.1 years. Of 21 patients followed for more than 2 years, 19 (90 %) achieved clinical remission, and 16 (76 %) achieved a state of maintained clinical remission on low-dose prednisone. Three patients developed proliferative nephritis on subsequent renal biopsy. Lower albumin at the time of biopsy was correlated with a lower rate of remission and longer time to remission., Conclusions: Among our paediatric patient cohort the outcome of non-proliferative MLN in systemic lupus erythematosus was good. The majority of patients did not require aggressive immunosuppressive treatment to reach a stable disease state on low-dose steroid treatment.

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a severe but potentially reversible neurologic disorder that is clinically recognizable in children and adolescents. Prompt diagnosis and treatment are essential to facilitate recovery. Treatment consists of corticosteroids, intravenous immunoglobulin, or plasma exchange as first-line therapy followed by cyclophosphamide or rituximab, if necessary, as second-line immunotherapy. Patients with tumor-associated encephalitis benefit from tumor resection. More than 75% of patients make a substantial recovery, which occurs in the reverse order of symptom presentation associated with a decline in antibody titers., (Crown Copyright © 2013. Published by Elsevier Inc. All rights reserved.)

Inflammatory brain diseases in childhood are underrecognized and lead to devastating yet potentially reversible deficits. New-onset neurologic or psychiatric deficits in previously healthy children mandate an evaluation for an underlying inflammatory brain disease. Distinct disease entities, such as central nervous system (CNS) vasculitis, are now being increasingly reported in children. Clinical symptoms, initial laboratory test, and neuroimaging studies help to differentiate between different causes; however, more invasive tests, such as lumbar puncture, conventional angiography, and/or brain biopsy, are usually necessary before the start of treatment. This article focuses on childhood CNS vasculitis., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Objectives: (1) To identify the average lumbar spine (LS) bone mineral density (BMD) trajectory in paediatric systemic lupus erythematosus (pSLE) patients, and (2) to identify predictors of BMD trajectory., Methods: 68 consecutive newly diagnosed pSLE patients prospectively followed in our lupus cohort with three annual dual energy x-ray absorptiometry (DEXA) examinations were studied. Low LS BMD was defined as z-score ≤-2.0. Baseline and longitudinal clinical features including disease activity, treatment and bone physiology markers were collected. Hierarchical linear modelling was used to model trajectory of LS BMD and identify predictors., Results: Women constituted 84% of the cohort and median age at diagnosis was 13.1 years. The mean LS BMD z-scores decreased over time (-0.42 at first, -1.02 at second and -1.11 at third DEXA). Initially 9% of patients had a low BMD, which increased to 19% by 3 years after diagnosis. 35% of patients deteriorated in BMD category from the first to third DEXA. LS BMD (adjusted by height-for-age z-score) followed a general deteriorating trajectory of -0.06 z-score/year from diagnosis. Increased rate of deterioration of BMD trajectory was predicted by pubertal status at diagnosis, increased interval cumulative steroid exposure and decreased weight z-scores., Conclusions: The LS BMD of pSLE patients followed a general deteriorating trend over time and could be predicted by a combination of pubertal status at diagnosis, interval cumulative doses of steroids and weight z-scores. Interval cumulative steroid dose represents an important target that clinicians may modify to ameliorate deteriorating BMD trajectory over time.

Objective: Muckle-Wells syndrome (MWS) is an inherited autoinflammatory disease caused by mutations in the NLRP3 gene that result in excessive interleukin-1 (IL-1) release. It is characterized by severe fevers, rashes, arthralgia, and conjunctivitis, leading to sensorineural deafness and amyloidosis. The recombinant IL-1 receptor antagonist anakinra blocks the biologic activity of IL-1. The aim of this study was to determine the short- and long-term efficacy and safety of anakinra therapy in children and adults with severe MWS., Methods: A single-center observational study was performed. Standardized assessments included clinical features, the Disease Activity Score (DAS) for MWS, classic and novel markers of inflammation, and patient-derived measures of health status. The primary outcome was a score of <10 on the DAS for MWS at 2 weeks and at the last followup visit. Measures of MWS disease activity were investigated using descriptive statistics and paired comparative analysis., Results: A total of 12 patients with severe MWS (5 children and 7 adults) received anakinra for a median of 11 months (range 5-14 months). The median followup was 11 months (range 5-14 months). Disease activity was significantly lower in all patients at 2 weeks (P = 0.0005). Organ manifestations of MWS improved, as did all patient-derived measures of health status, markers of inflammation, and hearing loss in 2 of the patients. Levels of the novel neutrophil activation biomarker S100A12 followed clinical disease activity. Treatment was well tolerated, and no serious adverse events were observed., Conclusion: Anakinra was found to be a safe and effective treatment of severe MWS, leading to a significant improvement in disease activity at 2 weeks as well as long-term. Anakinra therapy should therefore be considered in children and adults with severe MWS disease requiring IL-1 blockade., (Copyright © 2011 by the American College of Rheumatology.)

Objective: To determine the feasibility of conducting a randomized controlled trial of a 12-week exercise intervention in children with fibromyalgia (FM) and to explore the effectiveness of aerobic exercise on physical fitness, function, pain, FM symptoms, and quality of life (QOL)., Methods: FM patients ages 8-18 years were randomized to a 12-week exercise intervention of either aerobics or qigong. Both groups participated in 3 weekly training sessions. Program adherence and safety were monitored at each session. Data were collected at 3 testing sessions, 2 prior to and 1 after the intervention, and included FM symptoms, function, pain, QOL, and fitness measures., Results: Thirty patients participated in the trial. Twenty-four patients completed the program; 4 patients dropped out prior to training and 2 dropped out of the aerobics program. Better adherence was reported in the aerobics group than in the qigong group (67% versus 61%). Significant improvements in physical function, functional capacity, QOL, and fatigue were observed in the aerobics group. Anaerobic function, tender point count, pain, and symptom severity improved similarly in both groups., Conclusion: It is feasible to conduct an exercise intervention trial in children with FM. Children with FM tolerate moderate-intensity exercise without exacerbation of their disease. Significant improvements in physical function, FM symptoms, QOL, and pain were demonstrated in both exercise groups; the aerobics group performed better in several measures compared with the qigong group. Future studies may need larger sample sizes to confirm clinical improvement and to detect differences in fitness in childhood FM.

Objective: Lipid abnormalities in patients with systemic lupus erythematosus (SLE) are common and likely are one of the causes of premature atherosclerosis in these patients. Our aims were to determine the frequency and pattern of dyslipoproteinemia at presentation of pediatric SLE; and to determine the association between dyslipoproteinemia and markers of disease activity and inflammatory markers at presentation of pediatric SLE., Methods: Serum lipid measurements were obtained at diagnosis before corticosteroid treatment for an inception cohort of 54 patients. Total cholesterol, triglyceride, LDL-C, and HDL-C levels were regressed on measures of inflammation, disease activity, and disease symptoms., Results: At least one lipid abnormality was present in the majority of patients (63%), an elevated triglyceride level being the most common lipid abnormality (62%). Triglycerides were best predicted by fibrinogen, nephritis, and pleuritis (model R2 = 0.6). Albumin, C4, and white blood cell count were found to predict HDL-C (model R2 = 0.6). Erythrocyte sedimentation rate, central nervous system involvement, nasal ulcers, and nephritis were found as predictors for LDL-C:HDL-C (model R2 = 0.5). No significant predictors were found for total cholesterol or LDL-C. The European Consensus Lupus Activity Measure disease activity score best predicted abnormal triglyceride and HDL-C levels (OR 1.7, 95% CI 1.2-2.3)., Conclusion: Children with newly diagnosed SLE exhibited the distinct pattern of dyslipoprotein of increased triglycerides and depressed HDL-C that was twice as common in the presence of kidney disease. This lipid profile puts them at risk for premature atherosclerosis. Good disease control and individualized use of lipid-lowering agents based on the observed pattern of lipid abnormalities may lower the risk of premature atherosclerosis in these patients.

Central nervous system (CNS) vasculitis is an increasingly recognized, often devastating inflammatory brain disease of children and adults. In primary or isolated CNS vasculitis/angiitis of childhood (cPACNS), the vascular inflammation is limited to the brain and spinal cord. Secondary CNS vasculitis occurs in a variety of conditions including infections, collagen vascular diseases, systemic vasculidities, and malignancies. Mimics of CNS vasculitis in children include dissections and noninflammatory vasculopathies. Diagnosis of primary CNS vasculitis in both adults and children is based on the Calabrese criteria. This review summarizes recent data on CNS vasculitis in children; reviews the clinical spectrum at presentation and the role of laboratory tests, neuroimaging, and brain biopsy; and discusses treatment strategies, outcome data, and overlapping conditions of cPACNS.