Consideration concerning similarities and differences between ANCA-associated vasculitis and IgG-4-related diseases: case series and review of literature

Immunol Res. 2019 Feb;67(1):99-107. doi: 10.1007/s12026-019-9070-7.


Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and immunoglobulin G4-related diseases (IgG4-RD) are regarded as entirely different disease types with different etiological mechanisms. However, we experienced two cases that had clinical features of both AAV and IgG4-RD. The first case is an 81-year-old woman who showed periaortitis and retroperitoneal fibrosis and periarteritis with elevation of myeloperoxidase-anti-neutrophil cytoplasmic antibody and IgG4 levels. The second case is a 63-year-old woman who had dura mater, ear, nose, lung, and kidney involvement with serum negative for ANCA and elevated IgG4. Renal biopsy revealed tubulointerstitial nephritis involving IgG4+ plasma cells (IgG4+/IgG+ cell ratio of ≥ 40%). On the other hand, lung biopsy showed features of granulomatosis with polyangiitis (GPA). These two cases suggested that AAV and IgG4-RD might overlap. To investigate the similarities and differences between AAV and IgG4-RD, we retrospectively analyzed 13 cases of typical GPA, a subtype of AAV, and 13 cases of typical IgG4-RD at our hospital for comparison of clinical features and found some differences that can be useful in the differential diagnosis between the two diseases. Although AAV and IgG4-RD are distinguishable based on characteristic findings in many cases, the diagnosis can be unclear in rare cases, in which clinicians should consider possible coexistence of AAV and IgG4-RD when performing further workup. Here, we discuss the similarities and differences between AAV and IgG4-RD on the basis of our results and past literature.

Keywords: ANCA-associated vasculitis; Granulomatosis with polyangiitis; IgG4-related disease; Retroperitoneal fibrosis; Similarity and difference.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged, 80 and over
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / diagnosis*
  • Autoantibodies / metabolism
  • Diagnosis, Differential
  • Female
  • Humans
  • Immunoglobulin G / metabolism*
  • Immunoglobulin G4-Related Disease / diagnosis*
  • Kidney
  • Middle Aged
  • Plasma Cells / immunology*
  • Retrospective Studies


  • Autoantibodies
  • Immunoglobulin G