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Journal of Nephrology

, Volume 27, Issue 2, pp 159–164 | Cite as

Clinico-pathological features and outcomes of patients with propylthiouracil-associated ANCA vasculitis with renal involvement

  • Yinghua Chen
  • Hao Bao
  • Zhengzhao Liu
  • Haitao Zhang
  • Caihong Zeng
  • Zhihong Liu
  • Weixin Hu
Original Article

Abstract

Objective

To retrospectively investigate clinico-pathological features and outcomes of patients with renal involvement in propylthiouracil (PTU)-associated antineutrophil cytoplasmic autoantibody (ANCA) vasculitis (PTU-AAV).

Methods

Clinico-pathological features and outcomes of 12 patients (female 11, average age 32.4 ± 13.8 years) who developed AAV after treatment with PTU were collected and analyzed. ANCA was detected by both immunofluorescence (IF) and enzyme linked immunosorbent assay (ELISA). All patients had renal biopsy.

Results

Twelve patients received PTU for 2–264 months (median 42 months) when PTUAAV was diagnosed. All patients had positive serum P-ANCA, 11 of them were MPO-ANCA, 1 was MPO and PR3-ANCA double positive. All patients presented with hematuria and proteinuria, 5 of them had gross hematuria, urine protein was 1.9 ± 1.6 g/24 h, 7 of 12 (58.3 %) patients had renal dysfunction, among them 3 needed initial renal replacement therapy. Renal biopsy showed pauci-immune segmental necrotizing crescentic glomerulonephritis in ten patients, segmental necrotizing glomerulonephritis superimposed on membranous nephropathy in two patients. All patients withdrew PTU and received steroid and immunosuppressive therapy. After a median follow-up of 42 months (range 21–86), 3 patients developed to ESRD, 7 patients entered complete renal remission. Serum ANCA turned negative only in 2 patients, 10 patients had persistent positive serum ANCA. Three patients relapsed with the elevation of serum ANCA level.

Conclusion

Renal damage of PTU-AAV could be pauci-immune necrotizing crescentic glomerulonephritis, and necrotizing glomerulonephritis coexisted with membranous nephropathy. Most patients had persistent positive serum ANCA and had a risk of relapse and progression to ESRD even after PTU withdrawl and immunosuppressive therapy.

Keywords

Propylthiouracil (PTU) Antineutrophil cytoplasmic autoantibody (ANCA) Vasculitis 

Notes

Acknowledgments

This work was supported by the National Key Technology R&D Programm (2011BAI10B04).

Conflict of interest

The authors have no conflict of interest or financial relationships to disclose.

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Copyright information

© Italian Society of Nephrology 2014

Authors and Affiliations

  • Yinghua Chen
    • 1
  • Hao Bao
    • 1
  • Zhengzhao Liu
    • 1
  • Haitao Zhang
    • 1
  • Caihong Zeng
    • 1
  • Zhihong Liu
    • 1
  • Weixin Hu
    • 1
  1. 1.National Clinical Research Center of Kidney Diseases, Jinling HospitalUniversity School of MedicineNanjingChina

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