Fig. 3

Clinical course of the patient. After the diagnosis of TAFRO syndrome, steroid pulse therapy (500 mg/day of intravenous methylprednisolone) was initiated for 3 days from the 11th hospital day. Thereafter, 40 mg/day of prednisolone was administered orally. However, she developed anasarca, renal dysfunction, and oliguria. Hemodialysis was required from the 15th hospital day. Moreover, serum CRP level remained high, and she experienced considerable painful; therefore, treatment with an anti-IL-6 receptor antibody (tocilizumab) was started at a dose of 8 mg/kg (400 mg/day). Her pain reduced considerably; there was gradual improvement in her condition with respect to renal function and edema. Tocilizumab was administered again after 2 weeks. There was an increase in the urinary volume about 2 weeks after the tocilizumab therapy, and hemodialysis was discontinued. Serum VEGF and IL-6 levels after the second tocilizumab therapy were lower at 39.6 pg/mL (normal < 38.3) and 110 pg/mL (normal < 8), respectively. After the dose of prednisolone was tapered to 35 mg/day, 150 mg/day of cyclosporine was administered orally. However, oral cyclosporine was stopped because of adverse effects such as liver dysfunction and vomiting. She was discharged on hospital day 58. Abbreviations: BW: body weight; Cre: Creatinine; CRP: C-reactive protein; CyA: cyclosporine; IL: Interleukin; m-PSL: methylprednisolone; Plt: platelets; PSL: Prednisolone; TCZ: tocilizumab; VEGF: Vascular endothelial growth factor