Long term treatment with infliximab in pediatric Vogt-Koyanagi-Harada disease.

Purpose: To report a case of pediatric Vogt-Koyanagi-Harada (VKH) successfully treated with infliximab and methotrexate for ten years.

Observations: A 9-year-old Hispanic girl with VKH disease, was successfully treated with oral methotrexate 15 mg/week and oral prednisone 40 mg/day (1mg/kg/day). But when oral prednisone was tapered to 10 mg/day over a 3-month period, inflammation recurred.

[Subglottic stenosis as the initial manifestation of Wegener's granulomatosis in a teenager. Case report].

Wegener's granulomatosis is a necrotizing granulomatous vasculitis of autoimmune origin that primarily affects the upper and lower airways and kidneys. It is very rare in children and adolescents. When started at a young age it is often associated with subglottic stenosis.

Agreement between multi-dimensional and renal-specific response criteria in patients with juvenile systemic lupus erythematosus and renal disease.

Objectives: To evaluate change over time and level of agreement of renal-specific and multi-dimensional measures in juvenile systemic lupus erythematosus (SLE) with renal disease.

Methods: An analysis was made of 205/557 children with baseline 24-hour proteinuria >or=0.5 g.

Macrophage activation syndrome in juvenile systemic lupus erythematosus: a multinational multicenter study of thirty-eight patients.

Objective: To describe the clinical and laboratory features of macrophage activation syndrome as a complication of juvenile systemic lupus erythematosus (SLE).

Methods: Cases of juvenile SLE-associated macrophage activation syndrome were provided by investigators belonging to 3 pediatric rheumatology networks or were found in the literature. Patients who had evidence of macrophage hemophagocytosis on bone marrow aspiration were considered to have definite macrophage activation syndrome, and those who did not have such evidence were considered to have probable macrophage activation syndrome.

Macrophage activation syndrome in juvenile systemic lupus erythematosus: an under-recognized complication?

Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic diseases that is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to widespread haemophagocytosis and cytokine overproduction. It is seen most commonly in systemic juvenile idiopathic arthritis, but is increasingly recognized also in juvenile systemic lupus erythematosus (J-SLE). Recognition of MAS in patients with J-SLE is often challenging because it may mimic the clinical features of the underlying disease or be confused with an infectious complication.