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Biopsy-proven kidney involvement in hypocomplementemic urticarial vasculitis.

Alice Corthier1, Marie Jachiet2, Daniel Bertin3

  • 1Department of Nephrology, Aix-Marseille Univ, Hôpital de la Conception, AP-HM, 147 Bd Baille, 13005, Marseille, France.

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Summary

Hypocomplementemic urticarial vasculitis (HUV) can cause severe kidney problems, including acute kidney injury and end-stage kidney disease. Kidney biopsies often reveal glomerulonephritis or vasculitis, sometimes without anti-C1q antibodies.

Keywords:
Anti-C1q antibodyC1q depositsGlomerulonephritisHypocomplementemic urticarial vasculitisMcDuffie syndromeRenal biopsyRenal vasculitis

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Area of Science:

  • Nephrology
  • Rheumatology
  • Immunology

Background:

  • Hypocomplementemic urticarial vasculitis (HUV) is a rare systemic vasculitis.
  • Kidney involvement in HUV is not well-characterized.

Purpose of the Study:

  • To describe kidney involvement in HUV using a multicenter national cohort.
  • To analyze long-term outcomes of renal manifestations in HUV.

Main Methods:

  • Retrospective analysis of HUV patients with biopsy-proven kidney involvement.
  • Included patients identified through the French Vasculitis Study Group (FVSG).
  • Performed a systematic literature review on HUV kidney involvement.

Main Results:

  • Twelve HUV patients with kidney involvement were analyzed; 8 had positive anti-C1q antibodies.
  • Common kidney biopsy findings included membrano-proliferative glomerulonephritis (MPGN) and crescentic glomerulonephritis (GN).
  • Significant renal dysfunction, including acute kidney injury (AKI) and end-stage kidney disease (ESKD), was observed in several patients.

Conclusions:

  • Renal involvement in HUV can lead to severe kidney damage, including AKI, chronic kidney disease (CKD), and ESKD.
  • Anti-C1q antibodies are not always present in HUV with renal involvement.
  • Kidney biopsy findings are diverse, frequently showing MPGN or crescentic GN with C1q deposits.