Fig. 5

Longitudinal serum analyses before and after introduction of etoposide treatment in patient #3. The progression of plasma HMGB1 levels is illustrated in a. The first two plasma samples were collected at onset of sJIA without MAS manifestations. The HMGB1 levels increased at onset of MAS and declined promptly after treatment with etoposide infusions. Increased serum concentrations of ferritin (b) and IFN-γ (c) were documented during active MAS followed by a normalization post etoposide treatment. Serum IL-18 levels (d) were distinctly increased during the entire observation period with peak values during active phases of MAS. Serum MCP-1 levels did not reflect the clinical course (e). CsA: cyclosporine A; MP-pulses: methylprednisolone pulses