Kidney Week

Abstract: SA-PO1080

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

Session Information

• Pathology and Lab Medicine: Clinical
  October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Pathology and Lab Medicine

• 1502 Pathology and Lab Medicine: Clinical

Authors

• Hara, Akinori, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan

Akinori Hara,

• Tran, Trang Thi thu, Kanazawa University, KANAZAWA, Ishikawa, Japan

Trang Thi thu Tran,

• Kitagawa, Kiyoki, Kanazawa Medical Center, Kanazawa, ISHIKAWA, Japan

Kiyoki Kitagawa,

• Kitajima, Shinji, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan

Shinji Kitajima,

• Toyama, Tadashi, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan

Tadashi Toyama,

• Iwata, Yasunori, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan

Yasunori Iwata,

• Sakai, Norihiko, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan

Norihiko Sakai,

• Shimizu, Miho, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan

Miho Shimizu,

• Furuichi, Kengo, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan

Kengo Furuichi,

• Wada, Takashi, Division of Nephrology, Kanazawa University Hospital, Kanazawa, ISHIKAWA, Japan

Takashi Wada,

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.