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ANCA-associated kidney disease preceded by orbital pseudotumor.
- Source :
-
Pediatric Nephrology . Mar2024, Vol. 39 Issue 3, p741-744. 4p. - Publication Year :
- 2024
-
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. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 0931041X
- Volume :
- 39
- Issue :
- 3
- Database :
- Academic Search Index
- Journal :
- Pediatric Nephrology
- Publication Type :
- Academic Journal
- Accession number :
- 175022085
- Full Text :
- https://doi.org/10.1007/s00467-023-06172-w