Mycophenolate mofetil treatment in two children with severe polyarteritis nodosa refractory to immunosuppressant drugs.

Polyarteritis nodosa (PAN) is a necrotizing vasculitis of small- and medium-sized arteries with multiorgan involvement, rarely reported in childhood. Despite aggressive treatment with the combination of corticosteroids and cytotoxic agents, the overall prognosis is poor in most patients. We report on two siblings, now 15- and 14-year-old, affected with childhood onset PAN, refractory to multiple therapies, who showed rapid clinical and laboratory improvement when mycophenolate mofetil was introduced.

Kawasaki syndrome: an intriguing disease with numerous unsolved dilemmas.

More than 40 years have passed since Kawasaki syndrome (KS) was first described. Yet KS still remains an enigmatic illness which damages the coronary arteries in a quarter of untreated patients and is the most common cause of childhood-acquired heart disease in developed countries. Many gaps exist in our knowledge of the etiology and pathogenesis of KS, making improvements in therapy difficult.

Cross-sectional and longitudinal evaluation of bone mass in children and young adults with juvenile idiopathic arthritis: the role of bone mass determinants in a large cohort of patients.

Objective: To assess the prevalence of reduced spine bone mineral apparent density (BMAD), and to identify the main predictors of reduced spine BMAD in a cross-sectional and longitudinal evaluation of the same large cohort of patients with juvenile idiopathic arthritis (JIA). There are few prospective data on bone mass evaluation in a large number of patients with JIA, and with enthesitis-related arthritis onset.

Methods: Two hundred nineteen patients with JIA (median age 8.

Serum osteopontin as a predictive marker of responsiveness to methotrexate in juvenile idiopathic arthritis.

Objective: To evaluate if serum concentrations of osteopontin (OPN) at baseline in patients with juvenile idiopathic arthritis (JIA) represent a potential predictor of responsiveness to methotrexate (MTX).

Methods: At diagnosis, 60 children with active JIA received MTX in addition to nonsteroidal antiinflammatory drugs. After 12 months of MTX treatment, 30 patients were defined as responders; the 30 nonresponders received anti-tumor necrosis factor-alpha therapy (etanercept) in addition to MTX; this group was then enrolled for an additional 12-month study period.