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. Clinical and laboratory findings in patients with macrophage activation syndrome were contrasted with those of 2 control groups composed of patients with active juvenile SLE without macrophage activation syndrome. The ability of each feature to discriminate macrophage activation syndrome from active disease was evaluated by calculating sensitivity, specificity, and area under the receiver operating characteristic curve.Results. The study included 38 patients (20 with definite macrophage activation syndrome and 18 with probable macrophage activation syndrome). Patients with definite and probable macrophage activation syndrome were comparable with regard to all clinical and laboratory features of the syndrome, except for a greater frequency of lymphadenopathy, leukopenia, and thromDr Pringe is recipient of an Alpha Scholarship from the European Union (contract no. AML/B7-311/970666/II-0246-FI).

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. This review summarizes the characteristics of patients with J-SLE-associated MAS reported in the literature or seen by the authors and analyses the distinctive clinical, diagnostic and therapeutic issues that the occurrence of MAS may raise in patients with J-SLE.

PurposeTo report a case of pediatric Vogt-Koyanagi-Harada (VKH) successfully treated with infliximab and methotrexate for ten years.ObservationsA 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. Patient was considered as corticosteroid-dependent thus infliximab 7mg/kg/pulse was started on days 0, 15, 60 and every 60 days thereafter. Six months after, infliximab was increased to 10mg/kg/pulse as cells in the anterior chamber were still observed. After four months of treatment, ocular inflammation was fully controlled, oral prednisone was tapered to discontinuation over a period of 10 months and methotrexate was maintained at 15 mg/week. At 1-year follow up, infliximab was reduced to 6 mg/kg/pulse as patient remained stable on examination. After being treated for 3-years it was decided to discontinue infliximab however, 2 + anterior chamber cells recurred after a dose was skipped thus infliximab was restarted. After 10 years treatment with infliximab 6 mg/kg/pulse every 60 days and methotrexate 15 mg/week associated, no relapsing inflammatory episodes and resolution of physical features of Cushing's syndrome were observed.Conclusion and importanceCombined therapy of infliximab and methotrexate for up to 10 years was efficacious in this girl in controlling recurrent inflammation without associated side effects. To the best of our knowledge, this is the longest reported clinical follow up of a pediatric VKH case supporting the use of infliximab and methotrexate without steroids treatment.

Macrophage activation syndrome (MAS) is a life-threatening complication of systemic inflammatory disorders that is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to widespread hemophagocytosis and cytokine overproduction. Recent findings in hemophagocytic lymphohistiocytosis, a disease that is clinically very similar to MAS, highlight the possible pathogenetic role of a defective function of cytotoxic lymphocytes. Prompt diagnosis is important and recently preliminary diagnostic guidelines for the syndrome have been proposed. The recognition that MAS belongs to the secondary or reactive hemophagocytic syndromes has led to propose to rename it according to the contemporary classification of histiocytic disorders. Cyclosporine A has been found to be effective in patients with corticosteroid resistant MAS.

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