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Kidney Week

Abstract: TH-PO609

A Retrospective Observational Study of the ANCA-Negative Renal Vasculitis Cohort in the National Rare Kidney Disease Registry

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • O'Brien, Sorcha, Tallaght University Hospital, Dublin, Dublin, Ireland
  • Stoneman, Sinead, University College Cork, Cork, Cork, Ireland
  • Little, Mark Alan, The University of Dublin Trinity College, Dublin, Ireland
  • Egan, Allyson Catherine, Tallaght University Hospital, Dublin, Dublin, Ireland
Background

A recent single centre study reported that ANCA-negative pauci-immune glomerulonephritis (PIGN) presents in younger patients, with higher end-stage kidney disease (ESKD) risk, despite lacking differences in histopathology. Fewer extra-renal manifestations were observed. The aim of this study was to review ANCA negative vasculitis PIGN outcomes in the Irish Rare Kidney Disease Registry.

Methods

Of 616 patients with PIGN in the registry, 13 were ANCA-negative (defined as negative on anti-MPO and anti-PR3 immunofluorescence / ELISA), with PIGN confirmed on kidney biopsy. Data collection included demographics, biopsy results, management, and outcomes.

Results

The mean age was 56.8 years (SD 12.85). 4 patients had renal limited disease. Most common other symptom/ system involved included: constitutional (n=4) and ENT (n=4), muco-cutaneous/ophthalmic (n=2), chest (n=2), neurological (n=1), abdominal (n=1). The mean estimated glomerular filtration rate (eGFR) at diagnosis was 19.4ml/min/1.73m (SD 19.9). Mean creatinine was 423μmol/L (SD 259.9). 8 patients had UPCR documented, with the mean UPCR recorded as 543.2 (SD 747.3).
Applying the Berden score to 11 participants: 6 focal, 3 mixed, 1 crescentic, 1 sclerosed were observed. 10 of 13 patients received immunosuppression. Induction treatment included Methylprednisolone (n=7), Cyclophosphamide (n=7), Rituximab (n=2), oral prednisolone (n=3), and PLEX treatment (n=1). 9 patients were continued on maintenance treatment: oral prednisolone (n=6), Azathioprine (n=5), Mycophenolate Mofetil (n=3), Cyclophosphamide (n=1), Rituximab (n=1). 4 patients were on renal replacement therapy (RRT) at diagnosis, 1 patient developed end-stage kidney disease (ESKD). 10 patients achieved remission, 1 did not. 3 had documented relapse. 1 patient died within 5-years of diagnosis.

Conclusion

ANCA-negative vasculitis with renal involvement requires early detection and treatment to prevent kidney disease progression. Renal disease may occur as a single manifestation or as part of a multi--systemic presentation, underscoring the importance of organ surveillance. Further elucidation of the pathogenesis and prognostic markers of relapse in ANCA negative vasculitis, will play a key role in improving outcomes.