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Coexistence of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and IgA nephropathy

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Abstract

Co-occurrence of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and IgA nephropathy (IgAN) is extremely uncommon. To date, only a few case reports have described such patients. Here, we describe the clinical presentation, pathologic features, treatment response, and outcome data of five patients with the rare form of co-existing AAV and IgAN and compared the characteristics of these patients to AAV patients with pauci-immune glomerulonephritis (n = 10) and IgAN patients (n = 10) that were selected as controls by stratified random sampling. In addition, we summarize all the previously reported cases of AAV and IgAN. In total, including the current study, 16 AAV/IgAN overlap cases were reported. Our five patients with the coexistence of AAV and IgAN were younger than the ten AAV patients with pauci-immune glomerulonephritis (22.6 ± 8.2 years versus 48.9 ± 15.7 years, respectively, P = 0.004). Histologically, they had a significantly lower percentage of glomeruli with fibrous crescents compared with AAV patients (0.0% versus 4.0%, P = 0.038). Compared with ten IgAN patients, our five AAV/IgAN patients had higher levels of ESR (P = 0.032) and CRP (P = 0.031). After accepting treatment with a combination of steroid and immunosuppressants, all patients showed a positive response to therapy, except for one patient in our cohort and another previously reported patient. We described the clinical presentation, pathologic features, treatment response, and outcome data of five patients with overlapping AAV and IgAN. They had mild glomerular pathological lesions and a positive response to aggressive immunosuppressive therapy. They were quite similar to pauci-immune AAV patients in clinical features, except for younger age. They had a lower percentage of glomeruli with fibrous crescents compared with AAV patients. In contrast to IgAN patients, they had higher levels of ESR and CRP. The mechanism of the coexistence of IgAN and AAV needs further study.

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References

  1. Kitching AR, Anders HJ, Basu N, Brouwer E, Gordon J, Jayne DR, et al. ANCA-associated vasculitis Nat Rev Dis Primers. 2020;6(1):71. https://doi.org/10.1038/s41572-020-0204-y.

    Article  Google Scholar 

  2. Working Group of the International Ig ANN the Renal Pathology S, Roberts IS, Cook HT, Troyanov S, Alpers CE, et al. The Oxford classification of IgA nephropathy: pathology definitions, correlations, and reproducibility. Kidney Int. 2009;76(5):546–56. https://doi.org/10.1038/ki.2009.168.

    Article  Google Scholar 

  3. Schena FP, Nistor I. Epidemiology of IgA nephropathy: a global perspective. Semin Nephrol. 2018;38(5):435–42. https://doi.org/10.1016/j.semnephrol.2018.05.013.

    Article  Google Scholar 

  4. Allmaras E, Nowack R, Andrassy K, Waldherr R, Fvd Woude, Ritz E. Rapidly progressive IgA nephropathy with anti-myeloperoxidase antibodies benefits from immunosuppression. Clin Nephrol. 1997;48(5–1997):269.

    CAS  Google Scholar 

  5. O’Donoghue DJ, Nusbaum P, Noel LH, Halbwachs-Mecarelli L, Lesavre P. Antineutrophil cytoplasmic antibodies in IgA nephropathy and Henoch-Schönlein purpura. Nephrol Dial Transplant. 1992;7(6):534–8.

    Google Scholar 

  6. Yang YZ, Shi SF, Chen YQ, Chen M, Yang YH, Xie XF, et al. Clinical features of IgA nephropathy with serum ANCA positivity: a retrospective case-control study. Clin Kidney J. 2015;8(5):482–8. https://doi.org/10.1093/ckj/sfv078.

    Article  CAS  Google Scholar 

  7. Xie L, He J, Liu X, Tang S, Wang W, Li F, et al. Clinical value of systemic symptoms in IgA nephropathy with ANCA positivity. Clin Rheumatol. 2018;37(7):1953–61. https://doi.org/10.1007/s10067-017-3931-z.

    Article  Google Scholar 

  8. Stefan G, Terinte-Balcan G, Stancu S, Zugravu A, Gherghiceanu M, Mircescu G. IgA nephropathy with serum ANCA positivity: case series and literature review. Rheumatol Int. 2021;41(7):1347–55. https://doi.org/10.1007/s00296-021-04888-2.

    Article  CAS  Google Scholar 

  9. Bantis C, Stangou M, Schlaugat C, Alexopoulos E, Pantzaki A, Memmos D, et al. Is presence of ANCA in crescentic IgA nephropathy a coincidence or novel clinical entity? A case series. Am J Kidney Dis. 2010;55(2):259–68. https://doi.org/10.1053/j.ajkd.2009.09.031.

    Article  CAS  Google Scholar 

  10. Ma Y, Chen L, Xu Y, Han Q, Yu B, Zhao J, et al. The clinicopathologic characteristics and complement activation of antineutrophil cytoplasmic antibody-associated vasculitides with glomerular IgA deposition. Appl Immunohistochem Mol Morphol. 2020;28(10):e87–93. https://doi.org/10.1097/PAI.0000000000000819.

    Article  CAS  Google Scholar 

  11. Vrtovsnik F, Queffeulou G, Skhiri H, Nochy D, Walker F, Hayem G, et al. Simultaneous IgA nephropathy and Wegener’s granulomatosis–overlap or coincidence (the role of renal biopsy). Nephrol Dial Transplant. 1999;14(5):1266–7. https://doi.org/10.1093/ndt/14.5.1266.

    Article  CAS  Google Scholar 

  12. Richer C, Mouthon L, Cohen PPB, Royer I, Guettier C. IgA glomerulonephritis associated with microscopic polyangiitis or Churg-Strauss syndrome. Clin Nephrol. 1999;52(1):47–50.

    CAS  Google Scholar 

  13. Rao N, Bendall A, Lanteri M. ANCA vasculitis and IgA nephropathy linked to silica exposure. Occup Med (Lond). 2020;70(6):445–8. https://doi.org/10.1093/occmed/kqaa122.

    Article  CAS  Google Scholar 

  14. Shimizu M, Wada T, Izumiya NSY, Furuichi K, Kobayashi TMK, Goshima S. Clinicopathological features of antineutrophil cytoplasmic antibodiesassociated vasculitis in Japanese patients with IgA nephropathy. Clin Exp Nephrol. 2000;4:251–6.

    Article  CAS  Google Scholar 

  15. Joerg L, Kerstin A, Niko B, Dominik A, Martin K, Latus J. A typical Wegeners granulomatosis - but not pauci-immune! Minerva Urol Nefrol. 2012;64:149–52.

    CAS  Google Scholar 

  16. Kucuk A, Solak Y, Gaipov A, Bagcaci S, Esen H, Turk S, et al. Co-existing proteinase 3-antineutrophil cytoplasmic antibody-associated vasculitis with immunoglobulin A nephropathy. Korean J Intern Med. 2016;31(1):194–6. https://doi.org/10.3904/kjim.2016.31.1.194.

    Article  Google Scholar 

  17. Galante JR, Daruwalla CP, Roberts ISD, Haynes R, Storey BC, Bottomley MJ. An unusual presentation of propylthiouracil-induced anti-MPO and PR3 positive ANCA vasculitis with associated anti-GBM antibodies, IgA nephropathy and an IgG4 interstitial infiltrate: a case report. BMC Nephrol. 2020;21(1):295. https://doi.org/10.1186/s12882-020-01964-w.

    Article  CAS  Google Scholar 

  18. Fukuhara D, Kurayama R, Ito Y, Komagata Y, Arimura Y, Yan K. Granulomatosis with polyangiitis associated with IgA nephropathy. CEN Case Rep. 2013;2(2):204–8. https://doi.org/10.1007/s13730-013-0065-2.

    Article  Google Scholar 

  19. Berden AE, Ferrario F, Hagen EC, Jayne DR, Jennette JC, Joh K, et al. Histopathologic classification of ANCA-associated glomerulonephritis. J Am Soc Nephrol. 2010;21(10):1628–36. https://doi.org/10.1681/ASN.2010050477.

    Article  Google Scholar 

  20. Roberts IS. Pathology of IgA nephropathy. Nat Rev Nephrol. 2014;10(8):445–54. https://doi.org/10.1038/nrneph.2014.92.

    Article  CAS  Google Scholar 

  21. Rodrigues JC, Haas M, Reich HN. IgA nephropathy. Clin J Am Soc Nephrol. 2017;12(4):677–86. https://doi.org/10.2215/CJN.07420716.

    Article  CAS  Google Scholar 

  22. Wyatt RJ, Julian BA. IgA nephropathy. N Engl J Med. 2013;368(25):2402–14. https://doi.org/10.1056/NEJMra1206793.

    Article  CAS  Google Scholar 

  23. Kidney disease: improving global outcomes glomerular diseases work G. KDIGO 2021. Clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021;100(4S):S1–276. https://doi.org/10.1016/j.kint.2021.05.021.

    Article  Google Scholar 

  24. Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, et al. 2022 American College of Rheumatology/European alliance of associations for rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Ann Rheum Dis. 2022;81(3):309–14. https://doi.org/10.1136/annrheumdis-2021-221794.

    Article  Google Scholar 

  25. Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, et al. 2022 American College of Rheumatology/European alliance of associations for rheumatology classification criteria for granulomatosis with polyangiitis. Ann Rheum Dis. 2022;81(3):315–20. https://doi.org/10.1136/annrheumdis-2021-221795.

    Article  Google Scholar 

  26. Suppiah R, Robson JC, Grayson PC, Ponte C, Craven A, Khalid S, et al. 2022 American College of Rheumatology/European alliance of associations for rheumatology classification criteria for microscopic polyangiitis. Ann Rheum Dis. 2022;81(3):321–6. https://doi.org/10.1136/annrheumdis-2021-221796.

    Article  Google Scholar 

  27. Lin W, Shen C, Zhong Y, Ooi JD, Eggenhuizen P, Zhou YO, et al. Glomerular immune deposition in MPO-ANCA associated glomerulonephritis is associated with poor renal survival. Front Immunol. 2021;12:625672. https://doi.org/10.3389/fimmu.2021.625672.

    Article  CAS  Google Scholar 

  28. Haas M, Eustace JA. Immune complex deposits in ANCA-associated crescentic glomerulonephritis: a study of 126 cases. Kidney Int. 2004;65(6):2145–52. https://doi.org/10.1111/j.1523-1755.2004.00632.x.

    Article  CAS  Google Scholar 

  29. Wu T, Zhong Y, Zhou Y, Chen J, Yang Y, Tang R, et al. Clinical characteristics and prognosis in 269 patients with antineutrophil cytoplasimc antibody associated vasculitis. Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2020;45(8):916–22. https://doi.org/10.11817/j.issn.1672-7347.2020.190436.

    Article  Google Scholar 

  30. Wada T, Yokoyama H, Tomosugi N, Hisada Y, Ohta S, Naito T. Detection of urinary interleukin-8 in glomerular diseases. Kidney Int. 1994;46:455–60.

    Article  CAS  Google Scholar 

  31. Yokoyama H, Wada T, Furuichi K, Segawa C, Shimizu M, Kobayashi K. Urinary levels of chemokines (MCAF/MCP-1, IL-8) reflect distinct disease activities and phases of human IgA nephropathy. J Leukoc Biol. 1998;63:493–9.

    Article  CAS  Google Scholar 

  32. Yokoyama H, Takaeda M, Wada T, Ohta S, Hisada Y, Segawa C. Glomerular ICAM-1 expression related to circulating TNF-alpha in human glomerulonephritis. Nephron. 1997;76:425–33.

    Article  CAS  Google Scholar 

Download references

Funding

This work was funded by the National Key R&D Program of China (2020YFC2005000 to XX), the Key Research and Development Program of Hunan province (2020WK2008 to YZ), the Science and Technology Innovation Program of Hunan Province (2020RC5002 to JO), the Natural Science Foundation of Hunan Province (2021JJ31130 to YZ and 2020JJ6109 to CS), “Yiluqihang Shenmingyuanyang” Medical Development and Scientific Research Fund Project on Kidney Diseases (SMYY20220301001 to YZ), and Scientific Research Program of Traditional Chinese Medicine in Hunan Province (201761 to XL).

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Author notes

  1. Yong Zhong and Xiangcheng Xiao contributed equally to this work and share senior authorship.

Authors and Affiliations

  1. Department of Nephrology, Xiangya Hospital, Central South University, Changsha, Hunan Province, China

    Qi Xiong, Ting Meng, Rong Tang, Joshua D. Ooi, Wannian Nie, Xia Li, Qiaoling Zhou, Ping Xiao, Yong Zhong & Xiangcheng Xiao

  2. Key Laboratory of Biological, Nanotechnology of National Health Commission, Xiangya Hospital, Central South University, Changsha, Hunan, China

    Qi Xiong, Yong Zhong & Xiangcheng Xiao

  3. Department of Pathology, Xiangya Hospital, Central South University, Changsha, Hunan Province, China

    Wei Lin

  4. Department of Hematology, The Affiliated Zhuzhou Hospital Xiangya Medical College, Central South University, Zhuzhou, China

    Chanjuan Shen

  5. Centre for Inflammatory Diseases, Department of Medicine, Monash University, Monash Medical Centre, Clayton, VIC, Australia

    Joshua D. Ooi & Peter J. Eggenhuizen

  6. Department of Medical Records & Information, Xiangya Hospital, Central South University, Changsha, Hunan Province, China

    Jinbiao Chen

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  1. Qi Xiong
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Contributions

Y.Z., Q.X., R.T., T.M., W.L., and X.C.X. conceived and designed the research; Y.Z., Q.X., R.T., and X.C.X. wrote the paper; J.D.O., P.J.E., C.J.S., W.N.N., X.L., Q.L.Z., and P.X. revised the paper; Q.X., T.M., W.L., Y.Z., W.N.N., C.J.S., and X.L. collected the clinical and pathologic parameters of patients; Q.X., Y.Z., R.T., T.M., J.B.C, and W.L. analyzed data; all authors approved the final version of the manuscript.

Corresponding authors

Correspondence to Yong Zhong or Xiangcheng Xiao.

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The study was approved by the Medical Ethics Committee of the Xiangya Hospital of Central South University for Human Studies (approval number 201711836). Additional informed consent was obtained from all individual participants for whom identifying information is included in this article.

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Xiong, Q., Lin, W., Shen, C. et al. Coexistence of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and IgA nephropathy. Immunol Res 71, 1–14 (2023). https://doi.org/10.1007/s12026-022-09322-8

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