BLyS/APRIL dual inhibition for IgG4-RD: a prospective single-arm clinical trial of telitacicept

Figure 1 Clinical and laboratory parameters in IgG4-related disease (IgG4-RD) treated with telitacicept.(A) Left part shows previous IgG4-RD treatment strategy.The patient was diagnosed with IgG4-RD in Tongji Hospital in April 2017.At this point, the patient was treated with oral prednisone (30 mg orally) for 2 months.Prednisone dosage decreased regularly by 5 mg every 2 or 3 months to 5 mg in April 2018, which was maintained for 20 months until December 2019.And then withdrawal of prednisone sustained for 17 months until May 2021.(A) Right part shows serum levels of IgG (g/L), IgM (*10 g/L), IgE (*0.1 IU/L), IgA (g/L), IgG1-4 (g/L), C3 (g/L), C4 (g/L), Cr (µmol/L), eGFR (ml/min/1.73m 2 ) and IgG4-RD RI before and after treatment with telitacicept.Pink areas indicate the period of telitacicept treatment.Bar plot on the left and upper area shows the timeline of treatment from the first onset of IgG4-RD.(B) The maximum lesion area of left submandibular gland and lymph node in coronal plane, and the largest maximum lesion area of kidneys (red arrows) in transverse plane before and after 60 weeks of telitacicept treatment.(C) The safety data recorded during the telitacicept treatment.(D) Mann-Kendall trend analyses of the IgG4-RD RI, CD24-plasmablasts, IgE and IgM during the followup.(E) Principal component analysis of the baseline immune laboratory data of the enrolled cases.(F) Analysis of the coefficients of x-variables of the PC1's linear combination with the following baseline indices: gender, age, WBC, N#, L#, Mono#, Eos#, Baso#, ESR, hsCRP, IgG, IgA, IgM, C3, C4, IgG1, IgG2, IgG3, IgG4, IgE, IL-2, IL-4, IL-6, IL-10, TNF-α, IFN-γ, T cell, CD8+T cell, CD4+T cell, NK cell, B cell, Th/Ts.Data were analysed and plotted with Prism software (V.8.0.2) or R software (V.4.1.1).Baso#, absolute basophil count; Eos#, absolute eosinophil count; ESR, erythrocyte sedimentation rate; hsCRP, high-sensitive C-reactive protein; L#, absolute lymphocyte count; N#, absolute neutrophil count; Mono#, absolute monocyte count; RI, Responder index Index; WBC, white blood cell.