Abstract
Introduction: Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) and IgG4-related disease (IgG4-RD) are distinct immune disorders with overlapping clinical and laboratory features. While ANCA positivity excludes IgG4-RD in the 2019 ACR/EULAR classification, this criterion is not uniformly applied, and AAV can form inflammatory masses in various organs and show increase in IgG4 + plasma cells, similar to IgG4-RD. Case diagnosis/treatment: A 5-year-old female with history of orbital mass diagnosed as IgG4-RD presents with acute kidney injury. She has a myeloperoxidase ANCA, and kidney biopsy shows pauci-immune crescentic glomerulonephritis and acute tubulointerstitial nephritis with increased IgG4 + plasma cells and tubular basement membrane (TBM) deposits. Conclusion: In isolation, TBM deposits and increased IgG4 + plasma cells are suggestive of IgG4-RD. In the context of a positive ANCA and pauci-immune crescentic glomerulonephritis, however, increased IgG4 + plasma cells due to AAV are favored. In cases with features of IgG4-RD, ANCA positivity suggests an alternate diagnosis of AAV to be more likely.
Original language | English (US) |
---|---|
Pages (from-to) | 741-744 |
Number of pages | 4 |
Journal | Pediatric Nephrology |
Volume | 39 |
Issue number | 3 |
DOIs | |
State | Published - Mar 2024 |
Keywords
- ANCA
- Autoimmunity
- IgG4
- Nephritis
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Nephrology