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Table 1 (abstract O15). Clinical features of the VEXAS syndrome patients with RP using TCZ

From: Abstracts from the ISSAID 2021 Periodic Congress

Patient ID RP13 RP15 RP16
Age at diagnosis (years) 66.3 73.5 66.6
UBA1 variants p.Met41 c.122T>C: p.Met41Thr c.122T>C: p.Met41Thr c.121A>C: p.Met41Leu
Clinical findings at diagnosis High-grade fever, skin rash, RP, scleritis, peritonitis, pericarditis, meningitis High-grade fever, skin rash, RP, macrocytic anemia High-grade fever, skin rash, GCA, RP, MDS, DVT, scleritis, airway involvement
Treatments before TCZ PSL PSL, MTX PSL, AZP, colchicine
Symptoms existed at TCZ induction* High-grade fever, myalgia, headache Low-grade fever High-grade fever, skin rash, RBC and PLT transfusion-dependence
PSL dose before TCZ administration 9 mg 22.5 mg 30 mg
PSL dose at last visit 1 mg 12.5 mg 12.5 mg
Duration of TCZ administration at last visit 12.0 months 6.3 months 7.1 months
Symptoms existed after 6 months None None RBC transfusion-dependence, mild skin rash
  1. RP; relapsing chondritis, GCA; giant-cell arteritis, Hb; hemoglobin, DVT, deep vein thrombosis; MDS, myelodysplastic syndrome; PSL, prednisolone; MTX; methotrexate, AZP, azathioprine; TCZ, tocilizumab, RBC; red blood cells, PLT: platelets, * symptoms existed during the past 1 month before TCZ induction