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