Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2023 Aug 15:14:1227878.
doi: 10.3389/fimmu.2023.1227878. eCollection 2023.

An unsuspected histopathological finding -concomitant IgA nephropathy in a patient with ANCA-associated vasculitis: a case report and literature review

Maciej Tota  1 , Piotr Donizy  2 , Martyna Byrska  1 , Magdalena Krajewska  3 , Mariusz Kusztal  3
Affiliations
Review

An unsuspected histopathological finding -concomitant IgA nephropathy in a patient with ANCA-associated vasculitis: a case report and literature review

Maciej Tota et al. Front Immunol. .

Abstract

Although associations of IgA nephropathy (IgAN) and ANCA-associated vasculitis (AAV) have been described, this coexistence scarcely occurs and requires multidisciplinary management. Herein, we discuss a course of treatment introduced in a patient with two exacerbations. Furthermore, alterations in histopathological images between two kidney biopsies are presented. The applicability of traditional inflammatory markers, e.g., CRP, in monitoring disease severity in AAV and IgAN is limited. Based on our patient and current literature, we suggest ANCA testing in patients with rapidly progressing IgAN for therapeutic and prognostic purposes. As regards the therapy of IgAN associated with AAV, aggressive immunosuppressive regimens with methylprednisolone and cyclophosphamide are recommended. Alternatively, methylprednisolone with rituximab, plasma exchange, mycophenolate mofetil, and intravenous immunoglobulin (IVIG) could also be considered.

Keywords: ANCA-associated vasculitis; CRP; GPA; IgA nephropathy; PR-3 ANCA; granulomatosis with polyangiitis; kidney biopsy; treatment.

PubMed Disclaimer

Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
(A) First kidney biopsy. PAS staining of the cortex shows normal to mild mesangial hypercellularity with normal tubules and interstitium (100×). (B) First kidney biopsy. Immunohistochemistry for IgA reveals intense granular staining, predominantly mesangial (200×). (C) Second kidney biopsy. Glomerulus with fibrinoid necrosis and karyorrhexis. HE staining (400×). (D) Second kidney biopsy. Glomerulus with a cellular crescent. HE staining (600×). (E) Second kidney biopsy. Immunohistochemistry reveals a significant reduction of IgA reactivity in glomeruli (200×).
Figure 2
Figure 2
(A) Line graph showing the patient’s cANCA (PR3-ANCA) levels and proteinuria throughout therapy. The two exacerbations are marked with red lines (6 January 2019 and 30 June 2021), consistent with cANCA and proteinuria level elevation. PD, prednisone; MTX, methotrexate; AZT, azathioprine; CYC, cyclophosphamide; MTPD, methylprednisolone. (B) Line graph representing the patient’s CRP level and eGFR ratio changes.
Figure 3
Figure 3
Suggested association between COVID-19 infection and AAV development via neutrophil extracellular traps (NETs) formation.
See this image and copyright information in PMC

References

    1. D’Amico G. The commonest glomerulonephritis in the world: IgA nephropathy. Q J Med (1987) 64:709–27. - PubMed
    1. Tota M, Baron V, Musial K, Derrough B, Konieczny A, Krajewska M, et al. Secondary IgA nephropathy and IgA-associated nephropathy: A systematic review of case reports. J Clin Med (2023) 12:2726. doi: 10.3390/JCM12072726 - DOI - PMC - PubMed
    1. Suzuki H, Kiryluk K, Novak J, Moldoveanu Z, Herr AB, Renfrow MB, et al. The pathophysiology of IgA nephropathy. J Am Soc Nephrol (2011) 22:1795–803. doi: 10.1681/ASN.2011050464 - DOI - PMC - PubMed
    1. Kitching AR, Anders HJ, Basu N, Brouwer E, Gordon J, Jayne DR, et al. ANCA-associated vasculitis. Nat Rev Dis Prim (2020) 6:71. doi: 10.1038/S41572-020-0204-Y - DOI - PubMed
    1. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum (2013) 65:1–11. doi: 10.1002/ART.37715 - DOI - PubMed

MeSH terms