Lenalidomide for refractory cutaneous manifestations of pediatric systemic lupus erythematosus.

Objective Cutaneous manifestations of pediatric systemic lupus erythematosus cause significant morbidity. Lenalidomide, a thalidomide analogue, has shown promise treating cutaneous lupus erythematosus in adults. Our objective was to evaluate lenalidomide's efficacy and safety in treating refractory cutaneous manifestations of pediatric systemic lupus erythematosus. Methods We performed a retrospective chart review of 10 adolescents who received lenalidomide for recalcitrant cutaneous lupus erythematosus. Information was gathered at drug initiation and 6-month follow-up. The Wilcoxon matched-pairs signed-rank test was used to assess change in quantitative parameters of disease activity. Results Nine subjects were girls and six were African-American. Indications for lenalidomide treatment included alopecia, nasal and oral ulcers, extensive malar rash, discoid lesions, bullous lesions, panniculitis, cutaneous vasculitis, and Raynaud's phenomenon with digital ulcerations. Within 6 months, all patients demonstrated complete or near resolution based on physician report. Prednisone dose decreased from a mean 23.5 mg (SD± 13.3) to 12.25 mg (SD± 9.2) ( P= 0.008). Sedimentation rate decreased from a mean 29 mm/hour (SD± 31.5) to 17 mm/hour (SD± 18.1) ( P= 0.004). Lenalidomide was well tolerated. Conclusion Lenalidomide is an effective and safe treatment for a spectrum of dermatological conditions in pediatric systemic lupus erythematosus. Its use may allow a reduction in prednisone dose and decreased disfigurement. Prospective study is needed to clarify lenalidomide's role in treating cutaneous manifestations of systemic lupus erythematosus.

Long-term safety and effectiveness of etanercept in children with selected categories of juvenile idiopathic arthritis.

OBJECTIVE: This study was undertaken to evaluate the long-term safety and effectiveness of etanercept alone or in combination with methotrexate (MTX) in children with selected categories of juvenile idiopathic arthritis (JIA). METHODS: Patients ages 2-18 years with rheumatoid factor (RF)-positive or RF-negative polyarthritis, systemic JIA, or extended oligoarthritis were eligible for the study. Patients received MTX alone (> or =10 mg/m(2)/week [ approximately 0.3 mg/kg/week], maximum dosage 1 mg/kg/week), etanercept alone (0.8 mg/kg/week, maximum dose 50 mg), or etanercept plus MTX for 3 years in an open-label, nonrandomized study. Safety was assessed by measuring rates of adverse events, and effectiveness was assessed using the physician's global assessment of disease activity and the pediatric total joint assessment. RESULTS: A total of 197, 103, and 294 patients were enrolled in the MTX, etanercept, and etanercept plus MTX groups, respectively. Exposure-adjusted rates of adverse events were similar among the 3 treatment groups (18.3, 18.7, and 21.6 per 100 patient-years in the MTX, etanercept, and etanercept plus MTX groups, respectively). Respective rates per 100 patient-years of serious adverse events (4.6, 7.1, and 6.0) and medically important infections (1.3, 1.8, and 2.1) were also similar among the 3 treatment groups. Scores for physician's global assessment and total active joints improved from baseline, and improvement was maintained for the duration of the study. CONCLUSION: These data confirm the findings of other long-term studies and suggest that etanercept or etanercept plus MTX has an acceptable safety and effectiveness profile in children with selected categories of JIA. Improvement was maintained for 3 years in those continuing to receive medication.

Bone mineral status in juvenile rheumatoid arthritis.

Osteoporosis can be thought of as a disease of childhood with manifestations in the adult years. One strategy in prevention of osteoporosis is to maximize peak bone mass with interventions focused during the childhood and adolescent years, taking advantage of this unique window of opportunity to maximize bone mass accrual, maximize peak bone mass, and theoretically decrease fracture risk for life. Factors important in the development of peak bone mass in children are reviewed. Studies examining bone metabolism and bone density in children with juvenile rheumatoid arthritis (JRA) are summarized. There is much work to be done before the best treatments for the osteoporosis of JRA are defined. Optimizing calcium intake and physical activity, along with corticosteroid avoidance and control of disease activity, is sound management for children with JRA.

The school experience of children with arthritis. Coping in the 1990s and transition into adulthood.

To the arthritic child and his or her family, the pediatrician is a valuable resource. When school problems first occur in elementary school, the pediatrician can try to solve them with an informal contact with the school teacher, nurse, counselor, or principal. If that effort is unsuccessful, the pediatrician can then suggest that the family go to the school and request the Individual Education Plan (IEP). Although this mechanism is hardly a panacea and local school district variability exists, this process provides an opportunity for the parent and school personnel to sit down and discuss how the child's problems and illness impact school performance. Furthermore, the IEP attempts to adapt the school environment specifically for that child. The current mechanism of modification of a child's education is the result of a long, evolutionary societal and educational process. Educators now recognize that children with disabilities and chronic illnesses are entitled to an appropriate public education with a special focus on how illness can interfere with that education. Whatever congressional changes are likely in the 1990s, many of these civil rights advances for the disabled or chronically ill child should survive. The transition of the adolescent with a chronic illness, such as arthritis, into adulthood has received little attention. This is an area where a compassionate and supportive pediatrician may help prevent adolescent problems rather than simply reacting to them. A gradual, incremental transition program in the pediatric office and clinic may increase the chances that more of these special adolescents will become productive, contributing adults.

Breast feeding and the development of juvenile rheumatoid arthritis.

OBJECTIVE: To determine if children with juvenile rheumatoid arthritis (JRA) are less likely to have been breast fed than controls. METHODS: Case-control study of data obtained from a survey of mothers 54 children with JRA and 79 playmates regarding breast feeding. Duration of breast feeding was tabulated and odds ratios (OR) with 95% confidence intervals (CI) were determined. RESULTS: OR for breast feeding in children with JRA was 0.40 (0.20-0.81, 95% CI) compared to playmates. For pauciarticular JRA (N = 28) OR was 0.31 (0.10-0.93); in polyarticular JRA (N = 24) OR was 0.60 (0.21-1.70). Lower OR for increased durations of breast feeding were noted in children with JRA. CONCLUSION: Children who have had JRA, especially pauciarticular JRA, are less likely to have been breast fed than controls, suggesting that breast feeding may have a protective effect on the development of JRA.