Abstract Number: 293 • 2012 ACR/ARHP Annual Meeting
Usefulness of Cardiac Magnetic Resonance in the Assessment of Myocardial Inflammation and Fibrosis in Children Born to Mothers with Anti-SSA/Ro Antibodies: A Prospective Study of 23 Cases and 6 Controls
Background/Purpose: Besides congenital heart blocks (CHB), others manifestations of cardiac neonatal lupus erythematosus syndrome (NLES) include endocardial fibroelastosis (EFE), and dilated cardiomyopathy. Recently, autopsy of…Abstract Number: 294 • 2012 ACR/ARHP Annual Meeting
Effects of Obesity On Health-Related Quality of Life in Childhood-Onset Systemic Lupus Erythematosus
Background/Purpose: Obesity in adults with Systemic Lupus Erythematosus (SLE) is associated with an added risk of cardiovascular disease, decreased health-related quality of life (HRQOL), and…Abstract Number: 295 • 2012 ACR/ARHP Annual Meeting
Brain Biopsy Diagnosis in Magnetic Resonance Imaging Negative Childhood Primary Angiitis of the Central Nervous System
Background/Purpose: Primary CNS vasculitis in childhood (cPACNS) is a devastating inflammatory brain disease mandating rapid and accurate diagnostic evaluation, in order to optimize neurologic outcomes. …Abstract Number: 296 • 2012 ACR/ARHP Annual Meeting
A Brazilian Multicenter Study of 71 Children and Adolescents with Takayasu’s Arteritis
Background/Purpose: Takayasu’s arteritis is a chronic granulomatous disease that affects the vascular wall of the large arteries and can result in end organ damage. It…Abstract Number: 297 • 2012 ACR/ARHP Annual Meeting
Effectiveness of Intravenous Cyclophosphamide in Severe or Refractory Juvenile Dermatomyositis – A National Cohort Study: UK and Ireland
Background/Purpose: Juvenile dermatomyositis (JDM) is a rare autoimmune vasculopathy affecting primarily the muscle and skin, but can also involve other organs. Early and aggressive treatment improves outcome and prevents complications. Cyclophosphamide has been used as a second-line agent in the treatment of severe or refractory JDM. The published literature on the effectiveness of cyclophosphamide in JDM is limited to a small case series and case reports.The objective of the study is to describe the response to cyclophosphamide in the patients with severe or refractory JDM. Methods: 56 patients treated with cyclophosphamide between years 2000-2011 were identified in the JDM National (UK and Ireland) Cohort Biomarker Study and Repository for Idiopathic Inflammatory Myopathies. 8 patients were excluded due to incomplete data or too short follow up. For the 48 patients included, demographics, myositis core outcome variables, skin measures, laboratory measures, steroid dose and other treatments were recorded at baseline, time 6, 12, 18, 24 months and last follow up post commencement of the drug.Results: Indications for starting cyclophosphamide were ulcerative or severe skin disease, profound muscle weakness, lung disease, gastro-intestinal vasculopathy or refractory disease. Previous medications were steroids and Methotrexate for 47 patients and steroids and Cyclosporin for 1 patient. All patients starting with muscle weakness (n=44) significantly improved at time12, and the gains were maintained at follow up (see table1). Physician VAS was available for 32 patients and these all improved by 12 and 24 months, and for 31 remained stable at follow up. At last follow up 26/46(56%) had no rash, 32/46(69%) had normal nailfolds, 37/45(82%) had no Gottron’s, and calcinosis improved in 9/14(64%). Conclusion: This study, the largest to date, demonstrated significant improvement in both muscle and skin domains in patients with JDM treated with intravenous cyclophosphamide.Cyclophosphamide appears effective in the treatment of severe or refractory JDM.Table 1Table 2Abstract Number: 298 • 2012 ACR/ARHP Annual Meeting
Childhood-Onset Predicts Increased Disease Damage and Steroid Toxicity in a Cohort of Adults with Systemic Lupus Erythematosus
Background/Purpose: While previous work has shown that adults with childhood-onset systemic lupus erythematosus (cSLE) have increased risk of mortality, renal disease and myocardial infarction as…Abstract Number: 299 • 2012 ACR/ARHP Annual Meeting
Reduction of Cerebral and Corpus Callosum Volumes in Childhood-Onset Systemic Lupus Erythematosus. A Volumetric Magnetic Resonance Imaging Analysis
Background/Purpose: Cerebral atrophy has been described to occur in SLE with variable frequency. Aging, systemic diseases, corticosteroid use and central nervous system (CNS) involvement may…Abstract Number: 300 • 2012 ACR/ARHP Annual Meeting
Prevalence and Clinical Significance of Hippocampal Atrophy in Childhood-Onset Systemic Lupus Erythematosus
Background/Purpose: Hippocampal atrophy is associated with corticosteroid use and may be related to cognitive impairment in systemic lupus erythematosus (SLE).Objectives: To determine the prevalence of…Abstract Number: 301 • 2012 ACR/ARHP Annual Meeting
Decreased Frequency of Dystrophic Calcifications in Children with Juvenile Dermatomyositis: A 10-Year Study
Background/Purpose: In Juvenile Dermatomyositis (JDM), dystrophic calcifications, associated with increased morbidity and mortality, have been reported for 20-30% of patients. There are few laboratory indicators…Abstract Number: 302 • 2012 ACR/ARHP Annual Meeting
Accuracy of Systemic Lupus International Collaborating Clinics Classiffication Criteria Applied to Juvenile Systemic Lupus Erythematosus Patients
Background/Purpose: Systemic lupus erythematosus (SLE) is a prototype autoimmune disease. The most widely used classification criteria for SLE were those developed by the American College…Abstract Number: 303 • 2012 ACR/ARHP Annual Meeting
Ancestral Group Differences in Pediatric SLE Early Disease Severity: An Analysis of the Carranet Registry
Background/Purpose: Ancestral background may contribute to increased disease morbidity in patients with pediatric lupus systemic erythematosus (pSLE) of non-European descent; however, there exists a paucity…Abstract Number: 304 • 2012 ACR/ARHP Annual Meeting
Burden of Childhood Central Nervous System Vasculitis: Identifying High Risk Factors for Poor Cognitive Outcome
Background/Purpose: Childhood primary angiitis of the CNS (cPACNS) is an increasingly recognized, reversible cause of severe neurological and psychiatric deficit such as stroke, refractory seizures…Abstract Number: 305 • 2012 ACR/ARHP Annual Meeting
Clinicopathologic Correlates for Activity and Damage of Lupus Nephritis in Childhood-Onset Systemic Lupus Erythematosus
Background/Purpose: High AI activity (AI), tubulointerstitial (TI), and chronicity index (CI) scores from renal biopsy may predict poor renal outcomes in lupus nephritis (LN)…Abstract Number: 306 • 2012 ACR/ARHP Annual Meeting
Children with Probable SLE by ACR Criteria May Need More Aggressive Lupus Treatment Early in the Disease Course
Background/Purpose: This research explores whether delay of the childhood-onset SLE (cSLE) diagnosis until 4/11 ACR criteria are met affects patient outcome negatively Methods: Institutional Review…Abstract Number: 307 • 2012 ACR/ARHP Annual Meeting
Risk Factors for Poor Outcomes in Hospitalized Patients with Pediatric Systemic Lupus Erythematosus
Background/Purpose: Disparities in care among adults with SLE are well documented. We investigated associations of demographic factors and volume of annual inpatient hospital admissions with…