Figure 3

Longitudinal serum analyses before and after introduction of etoposide treatment in patient 2. Serum concentration of total HMGB1 and hyperacetylated HMGB1 are illustrated in (A) with the different HMGB1 redox-forms illustrated in (B). The first two serum samples were collected at onset of SoJIA without MAS manifestations when HMGB1 levels appeared mainly in its cytokine-inducing, hyperacetylated isoform. These HMGB1 levels increased at onset of MAS and declined promptly after treatment with etoposide infusions. Increased serum concentrations of (C) ferritin and (D) IFN-γ were documented during MAS followed by a normalization post etoposide treatment. (E) Serum IL-18 levels were distinctly increased during the entire observation period with peak values during MAS. (F) MCP-1 did not mimic the clinical course. MP-pulses: methylprednisolone pulses.