44. Differences in phenotype and treatment of GPA and MPA in Europe, Japan, and the US
Creators: name: Kida1, Takashi
name: Kronbichler3, Andreas
name: Duvuru Geetha4
name: Omura1, Satoshi
name: Ito-Ihara2, Toshiko
name: Kawahito1, Yutaka
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Background:</strong> There are known geographical differences in the epidemiology, clinical presentation, and management of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Currently, the pandemic of coronavirus disease 2019 (COVID-19) may provide additional uncertainty. In this retrospective observational study, we evaluated the phenotypes, treatments, and outcomes in Europe, Japan, and the United States (US) using contemporary cohorts of GPA and MPA. <strong>Methods</strong>: Patients with new-onset or severe relapse of GPA/MPA as classified by the Chapel Hill Consensus Conference classification between January 2020 and July 2020 were included in this study. Patients were enrolled from 7 sites in Europe (72 GPA, 65 MPA), 20 sites in Japan (19 GPA, 54 MPA), and 5 sites in the US (25 GPA, 19 MPA). We assessed baseline characteristics including demographics and clinical presentations, treatment regimens, and glucocorticoid doses in the three regions. End-stage renal disease (ESRD)-free survival at 1 year were compared using the adjusted survival curves. <strong>Results:</strong> There was no clear gender difference in GPA/MPA throughout the regions, and median age [interquartile range (IQR)] was 65 [57–73], 76 [71–82], and 70 [64–76] in Europe, Japan, and the US, respectively. For each region, patients were numerically older in MPA than GPA. Regarding atypical ANCA-status, that is, MPO-ANCA positivity in GPA (overall, 31 out of 116) was more prevalent in Japan (58%) and the US (44%) than in Europe (13%); PR3-ANCA positivity in MPA (overall, 16 out of 138) was more prevalent in Europe (15%) and the US (21%) than in Japan (3.7%). The frequency of lung involvement in GPA was consistent in each region, ranging from 52–60%, whereas those in MPA were more common in Japan (69%) than in Europe (39%) and the US (32%). The frequency of kidney involvement in GPA was inconsistent: 67% in Europe, 42% in Japan, and 88% in the US, whereas in MPA it was common in almost all patients in all regions. In Europe, Japan, and the US, the frequency of use of cyclophosphamide was 57%, 29%, and 34%, and that of rituximab was 63%, 40%, and 86%, the median oral prednisone dose (mg/day) was 40, 40, and 60 at the beginning and 5, 10, and 5 at 6 months, respectively. In Japan, 13 out of 73 patients (18%) were treated with glucocorticoid alone. The ESRD-free survival rates at 1 year in Europe, Japan, and the US were 78%, 80%, and 81%, respectively, calculated from the covariate-adjusted survival curves. <strong>Conclusions:</strong> Although the investigator's specialty should be considered, there are still regional differences in remission-induction regimens and glucocorticoid use, perhaps reflecting the diversity of clinical phenotypes of AAV. In the early stages of the COVID-19 pandemic, there was no apparent difference in ESRD-free survival among the three regions. <strong>Disclosures</strong>: TK received consulting fees from Chugai. AK received consulting fees from Otsuka, Vifor Pharma, UriSalt, Catalyst Biosciences, and Alexion. DG received consulting fees from ChemoCentryx and Aurinia. YK received research grants from Asahi-Kasei and Chugai, and consulting fees from Asahi-Kasei, Chugai, and Pfizer.