An unsuspected histopathological finding -concomitant IgA nephropathy in a patient with ANCA-associated vasculitis: a case report and literature review

Front Immunol. 2023 Aug 15:14:1227878. doi: 10.3389/fimmu.2023.1227878. eCollection 2023.

Abstract

Although associations of IgA nephropathy (IgAN) and ANCA-associated vasculitis (AAV) have been described, this coexistence scarcely occurs and requires multidisciplinary management. Herein, we discuss a course of treatment introduced in a patient with two exacerbations. Furthermore, alterations in histopathological images between two kidney biopsies are presented. The applicability of traditional inflammatory markers, e.g., CRP, in monitoring disease severity in AAV and IgAN is limited. Based on our patient and current literature, we suggest ANCA testing in patients with rapidly progressing IgAN for therapeutic and prognostic purposes. As regards the therapy of IgAN associated with AAV, aggressive immunosuppressive regimens with methylprednisolone and cyclophosphamide are recommended. Alternatively, methylprednisolone with rituximab, plasma exchange, mycophenolate mofetil, and intravenous immunoglobulin (IVIG) could also be considered.

Keywords: ANCA-associated vasculitis; CRP; GPA; IgA nephropathy; PR-3 ANCA; granulomatosis with polyangiitis; kidney biopsy; treatment.

Publication types

  • Review
  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / complications
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / diagnosis
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / drug therapy
  • Cyclophosphamide / therapeutic use
  • Glomerulonephritis, IGA* / diagnosis
  • Glomerulonephritis, IGA* / drug therapy
  • Humans
  • Methylprednisolone / therapeutic use
  • Plasma Exchange

Substances

  • Cyclophosphamide
  • Methylprednisolone

Grants and funding

This study was supported by the Wroclaw Medical University statutory funds (SUBZ.C160.23.046).