Abstract
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic inflammation of small or medium blood vessels that includes microscopic polyangiitis. A diagnosis of ANCA-associated vasculitis can be aided by histological identification of vasculitis, and identification of renal impairment can help predict outcomes. However, kidney biopsy is not generally indicated in the absence of renal findings. We report two cases of ANCA-associated vasculitis diagnosed by kidney biopsy despite the absence of remarkable urinary abnormality and renal impairment. These patients had fever of unknown origin and were positive for myeloperoxidase (MPO)-ANCA but showed few findings that would suggest small-vessel vasculitis in the kidney. Nevertheless, kidney biopsies revealed small-vessel arteritis, necrotizing glomerulonephritis, and interstitial nephritis. Immunofluorescent antibody tests performed using samples of glomeruli were all negative, suggesting microscopic polyangiitis. Therefore, kidney biopsy may be useful in confirming the diagnosis, even if patients have completely normal urinary findings in the absence of other organ lesions.
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs13730-022-00719-w/MediaObjects/13730_2022_719_Fig1_HTML.jpg)
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs13730-022-00719-w/MediaObjects/13730_2022_719_Fig2_HTML.jpg)
Similar content being viewed by others
References
Ntatsaki E, Watts RA, Scott DG. Epidemiology of ANCA-associated vasculitis. Rheum Dis Clin North Am. 2010;36:447–61.
Geetha D, Jefferson JA. ANCA-associated vasculitis: core curriculum 2020. Am J Kidney Dis. 2020;75:124–37.
Yates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016;75:1583–94.
Bajema IM, Hagen EC, Hermans J, et al. Kidney biopsy as a predictor for renal outcome in ANCA-associated necrotizing glomerulonephritis. Kidney Int. 1999;56:1751–8.
Hasegawa J, Hoshino J, Sekine A, et al. Clinical and pathological features of anti-neutrophil cytoplasmic antibody-associated vasculitis in patients with minor urinary abnormalities. Nephrology (Carlton). 2018;23:1007–12.
Houben E, van der Heijden JW, van Dam B, Bax WA, Voskuyl AE, Penne EL. Screening for renal involvement in ANCA-associated vasculitis: room for improvement? Neth J Med. 2017;75:21–6.
Mukhtyar C, Lee R, Brown D, et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis. 2009;68:1827–32.
Rhee RL, Davis JC, Ding L, et al. The utility of urinalysis in determining the risk of renal relapse in ANCA-associated vasculitis. Clin J Am Soc Nephrol. 2018;13:251–7.
Suppiah R, Robson JC, Grayson PC, et al. American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis. Arthritis Rheumatol. 2022;2022(74):400–6.
Robson JC, Grayson PC, Ponte C, et al. American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis. Arthritis Rheumatol. 2022;2022(74):393–9.
Grayson PC, Ponte C, Suppiah R, et al. American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Arthritis Rheumatol. 2022;2022(74):386–92.
Noone DG, Twilt M, Hayes WN, et al. The new histopathologic classification of ANCA-associated GN and its association with renal outcomes in childhood. Clin J Am Soc Nephrol. 2014;9:1684–91.
Chang DY, Wu LH, Liu G, Chen M, Kallenberg CG, Zhao MH. Re-evaluation of the histopathologic classification of ANCA-associated glomerulonephritis: a study of 121 patients in a single center. Nephrol Dial Transplant. 2012;27:2343–9.
Berden AE, Ferrario F, Hagen EC, et al. Histopathologic classification of ANCA-associated glomerulonephritis. J Am Soc Nephrol. 2010;21:1628–36.
Zoshima T, Suzuki K, Suzuki F, et al. ANCA-associated nephritis without crescent formation has atypical clinicopathological features: a multicenter retrospective study. Clin Exp Nephrol. 2020;24:999–1006.
Endo A, Hoshino J, Suwabe T, et al. Significance of small renal artery lesions in patients with antineutrophil cytoplasmic antibody-associated glomerulonephritis. J Rheumatol. 2014;41:1140–6.
Guchelaar NAD, Waling MM, Adhin AA, van Daele PLA, Schreurs MWJ, Rombach SM. The value of anti-neutrophil cytoplasmic antibodies (ANCA) testing for the diagnosis of ANCA-associated vasculitis, a systematic review and meta-analysis. Autoimmun Rev. 2021;20: 102716.
Poggio ED, McClelland RL, Blank KN, et al. Systematic review and meta-analysis of native kidney biopsy complications. Clin J Am Soc Nephrol. 2020;15:1595–602.
Cheung CK, Tan J, Lowe RA, Gouldesbrough D, Stoves J. ANCA-associated vasculitis complicated by haemoperitoneum. NDT Plus. 2010;3:142–4.
Chung SA, Langford CA, Maz M, et al. American College of Rheumatology/vasculitis foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2021;2021(73):1366–83.
Acknowledgements
We would like to thank the staff at the Departments of Rheumatology, Nephrology, and Pathology of the Kameda Medical Center for their dedicated effort in caring for these patients. We also thank Editage for English language editing.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors have declared that no conflict of interest exists.
Ethical statement
This article does not contain any studies with human participants or animals performed by any of the authors.
Informed consent
The patients’ families provided consent for the publication of this case report after the removal of all identifying information to retain the patients’ anonymity and privacy.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
About this article
Cite this article
Akao, S., Yamagiwa, G., Hazue, R. et al. Kidney biopsy in patients with antineutrophil cytoplasmic antibody-associated vasculitis with mild renal abnormality. CEN Case Rep 12, 50–55 (2023). https://doi.org/10.1007/s13730-022-00719-w
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s13730-022-00719-w