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. 2023 Mar 29;9(1):e002935. doi: 10.1136/rmdopen-2022-002935

Table 4.

Outcomes of patients with PR3-ANCA according with PRTN3 zygosity

PR3-AAV (n=64) Homozygous PRTN3-Val119
(n=51)		 Homozygous PRTN3-Ile119
(n=13)		 P value*
Remission, n (%) 45 (88.2) 13 (100) 0.194
Complete remission, n (%) 36 (70.6) 10 (76.9) 0.650
Any flare by 18 months, n (%) 30 (58.8) 7 (53.8) 0.746
Severe* flare by 18 months, n (%) 10 (19.6) 6 (46.2) 0.048

*Relapse was considered severe according with the RAVE trial definition: BVAS/WG >3 or new major item.

AAV, antineutrophil cytoplasmic antibody associated vasculitis; ANCA, antineutrophil cytoplasmic antibody; BVAS/WG, Birmingham Vasculitis Activity Score for Wegener Granulomatosis; n, number; PR3, proteinase 3; PRTN3, proteinase 3 gene; RAVE, Rituximab in ANCA-Associated Vasculitis.