Fig. 2

Schedule of enrolment, intervention, and assessment. a Gray shading shows the primary endpoint. b It is inferred experientially that CRP levels may become normal 3 days after IVIG completion when the patient switches from intravenous methylprednisolone to oral prednisolone tapered over 15 days. c Patients will accept rescue therapy if they exhibit IVIG resistance. d Axillary temperature (or rectal temperature) will be measured every 6 h from IVIG treatment. The time point, temperature, and treatment will be recorded if fever occurs between two measurements. When IVIG starts and body temperature becomes normal it will be recorded. e Laboratory examination includes CRP, ESR, Hct, Hb, ALB, SAA, prealbumin, ALT, AST, CK-MB, sodium, NT-proBNP, IL-2, IL-4, IL-6, IL-10, TNF-α, TB, troponin, D-dimer, lipid, and lipoprotein serum levels and WBC, NEUT, and PLT counts. CRP and routine blood tests will be measured every 3 days after completion of initial IVIG infusion until normal. The remaining indicators (except for ESR), if abnormal, will also be measured every 3 days after completion of initial IVIG infusion until normal. f Other auxiliary examination includes electrocardiogram, chest radiography, magnetic resonance angiography, and myocardial perfusion imaging. The patient will choose whether to accept the examinations based on the physical condition during the diagnostic and therapeutic period. ALB, serum albumin; ALT, alanine aminotransferase; AST, aspartate transaminase; CK-MB, creatine kinase-muscle/brain; CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; Hb, hemoglobin; Hct, hematocrit; IL-2, interleukin-2; IL-4, interleukin-4; IL-6, interleukin-6; IL-10, interleukin-10; IVIG, intravenous immunoglobulin; NEUT, neutrophil; NT-proBNP, N-terminal pro-B-type natriuretic peptide, PLT, platelet; SAA, serum amyloid A; TB, total bilirubin; TNF-α, tumor necrosis factor-alpha; WBC, white blood cell