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.
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References
Raissian Y, Nasr SH, Larsen CP, Colvin RB, Smyrk TC, Takahashi N, Bhalodia A, Sohani AR, Zhang L, Chari S, Sethi S, Fidler ME, Cornell LD (2011) Diagnosis of IgG4-related tubulointerstitial nephritis. J Am Soc Nephrol 22:1343–1352. https://doi.org/10.1681/ASN.2011010062
Wallace ZS, Naden RP, Chari S, Choi H, Della-Torre E, Dicaire JF, Hart PA, Inoue D, Kawano M, Khosroshahi A, Kubota K, Lanzillotta M, Okazaki K, Perugino CA, Sharma A, Saeki T, Sekiguchi H, Schleinitz N, Stone JR, Takahashi N, Umehara H, Webster G, Zen Y, Stone JH (2020) The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease. Arthritis Rheumatol 72:7–19. https://doi.org/10.1002/art.41120
Karim F, Loeffen J, Bramer W, Westenberg L, Verdijk R, van Hagen M, van Laar J (2016) IgG4-related disease: a systematic review of this unrecognized disease in pediatrics. Pediatr Rheumatol Online J 14:18. https://doi.org/10.1186/s12969-016-0079-3
Tsukikawa M, Lally SE, Shields CL, Eagle RC Jr, Ellis FJ, Wasserman BN (2019) Idiopathic orbital pseudotumor preceding systemic inflammatory disease in children. J Pediatr Ophthalmol Strabismus 56:373–377. https://doi.org/10.3928/01913913-20190923-02
Houghton DC, Troxell ML (2011) An abundance of IgG4+ plasma cells is not specific for IgG4-related tubulointerstitial nephritis. Mod Pathol 24:1480–1487. https://doi.org/10.1038/modpathol.2011.101
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Oleson, I., Fecker, A., Richardson, K. et al. ANCA-associated kidney disease preceded by orbital pseudotumor. Pediatr Nephrol 39, 741–744 (2024). https://doi.org/10.1007/s00467-023-06172-w
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DOI: https://doi.org/10.1007/s00467-023-06172-w