Fig. 3

Clinical course in Case 1 and Case 2. a In Case 1, treatment with PSL at a daily dose of 0.6 mg/kg (30 mg) was initiated. Because of no clinical response to PSL 0.6 mg/kg, the dose of PSL was increased to at a daily dose of 1.0 mg/kg (50 mg). Because intensifying the dose of PSL wasn’t effective, CyA treatment was induced. After beginning CyA treatment, her symptoms and laboratory data, including platelet count, markedly improved, and blood transfusion was no longer needed. b In Case 2 with the high serum IgG4 level, treatment with PSL at a daily dose of 0.6 mg/kg (40 mg) and total parenteral nutrition was initiated. After initiating PSL treatment, the serum IgG4 level decreased. However, clinical symptoms were exacerbated and nutrition condition didn’t improve. Therefore, CyA treatment was induced. After beginning CyA treatment, his symptoms and laboratory data markedly improved, and CyA treatment was continued orally at a trough level of 200 ng/ml. After beginning CyA treatment, the serum IgG4 level decreased from 638 mg/dl to 97.0 mg/dl