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Abstract: SA-PO1080

Effect of Autoantibodies to Erythropoietin Receptor in Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

Session Information

Category: Pathology and Lab Medicine

  • 1502 Pathology and Lab Medicine: Clinical

Authors

  • Hara, Akinori, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan
  • Tran, Trang Thi thu, Kanazawa University, KANAZAWA, Ishikawa, Japan
  • Kitagawa, Kiyoki, Kanazawa Medical Center, Kanazawa, ISHIKAWA, Japan
  • Kitajima, Shinji, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan
  • Toyama, Tadashi, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan
  • Iwata, Yasunori, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan
  • Sakai, Norihiko, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan
  • Shimizu, Miho, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan
  • Furuichi, Kengo, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan
  • Wada, Takashi, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan
Background

To examine the effect of autoantibodies to the erythropoietin receptor (EPOR) in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

Methods

Sixty-three Japanese patients with AAV were enrolled in this study and followed for a median of 31 months. Sera from these patients were screened for anti-EPOR antibodies using enzyme-linked immunosorbent assays.

Results

The patients comprised 26 men and 37 women aged 67±14 years. Fifteen patients had developed end-stage renal disease (ESRD) during follow-up period. Anti-EPOR antibodies were detected in 7 patients (11%). Anti-EPOR antibodies were associated with increased Birmingham vasculitis activity score (BVAS), especially systemic and skin scores. In addition, anti-EPOR antibodies were positively correlated with systemic and skin scores of BVAS. Cox regression analysis revealed that male gender, proteinuria and estimated glomerular filtration rate at disease onset were significant risk factors for ESRD. Among 6 patients who were positive for the antibodies at baseline and had follow-up examination of their sera, the antibodies disappeared in 4 patients by 2 months after immunosuppressive treatment, while remained positive in the other 2, and one of these developed ESRD.

Conclusion

Anti-EPOR antibodies were associated with disease activity, especially systemic manifestations and skin lesions in patients with AAV.