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

doi:10.1007/s12026-022-09322-8

. 2023 Feb;71(1):1-14.

doi: 10.1007/s12026-022-09322-8. Epub 2022 Oct 13.

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

Qi Xiong^{1

2}, Wei Lin³, Chanjuan Shen⁴, Ting Meng¹, Rong Tang¹, Joshua D Ooi^{1

5}, Peter J Eggenhuizen⁵, Jinbiao Chen⁶, Wannian Nie¹, Xia Li¹, Qiaoling Zhou¹, Ping Xiao¹, Yong Zhong^#^{7

8}, Xiangcheng Xiao^#^{9

10}

Affiliations

¹ Department of Nephrology, Xiangya Hospital, Central South University, Changsha, Hunan Province, China.
² Key Laboratory of Biological, Nanotechnology of National Health Commission, Xiangya Hospital, Central South University, Changsha, Hunan, China.
³ Department of Pathology, Xiangya Hospital, Central South University, Changsha, Hunan Province, China.
⁴ Department of Hematology, The Affiliated Zhuzhou Hospital Xiangya Medical College, Central South University, Zhuzhou, China.
⁵ Centre for Inflammatory Diseases, Department of Medicine, Monash University, Monash Medical Centre, Clayton, VIC, Australia.
⁶ Department of Medical Records & Information, Xiangya Hospital, Central South University, Changsha, Hunan Province, China.
⁷ Department of Nephrology, Xiangya Hospital, Central South University, Changsha, Hunan Province, China. zhongyong121@163.com.
⁸ Key Laboratory of Biological, Nanotechnology of National Health Commission, Xiangya Hospital, Central South University, Changsha, Hunan, China. zhongyong121@163.com.
⁹ Department of Nephrology, Xiangya Hospital, Central South University, Changsha, Hunan Province, China. xiaoxc@csu.edu.cn.
¹⁰ Key Laboratory of Biological, Nanotechnology of National Health Commission, Xiangya Hospital, Central South University, Changsha, Hunan, China. xiaoxc@csu.edu.cn.

^# Contributed equally.

PMID: 36227529
DOI: 10.1007/s12026-022-09322-8

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

Qi Xiong et al. Immunol Res. 2023 Feb.

. 2023 Feb;71(1):1-14.

doi: 10.1007/s12026-022-09322-8. Epub 2022 Oct 13.

Authors

Affiliations

¹ Department of Nephrology, Xiangya Hospital, Central South University, Changsha, Hunan Province, China.
² Key Laboratory of Biological, Nanotechnology of National Health Commission, Xiangya Hospital, Central South University, Changsha, Hunan, China.
³ Department of Pathology, Xiangya Hospital, Central South University, Changsha, Hunan Province, China.
⁴ Department of Hematology, The Affiliated Zhuzhou Hospital Xiangya Medical College, Central South University, Zhuzhou, China.
⁵ Centre for Inflammatory Diseases, Department of Medicine, Monash University, Monash Medical Centre, Clayton, VIC, Australia.
⁶ Department of Medical Records & Information, Xiangya Hospital, Central South University, Changsha, Hunan Province, China.
⁷ Department of Nephrology, Xiangya Hospital, Central South University, Changsha, Hunan Province, China. zhongyong121@163.com.
⁸ Key Laboratory of Biological, Nanotechnology of National Health Commission, Xiangya Hospital, Central South University, Changsha, Hunan, China. zhongyong121@163.com.
⁹ Department of Nephrology, Xiangya Hospital, Central South University, Changsha, Hunan Province, China. xiaoxc@csu.edu.cn.
¹⁰ Key Laboratory of Biological, Nanotechnology of National Health Commission, Xiangya Hospital, Central South University, Changsha, Hunan, China. xiaoxc@csu.edu.cn.

^# Contributed equally.

PMID: 36227529
DOI: 10.1007/s12026-022-09322-8

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.

Keywords: Antineutrophil cytoplasmic antibody; Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV); Autoimmune disease; Glomerular disease; IgA nephropathy.

PubMed Disclaimer

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 . - DOI
1. 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 . - DOI
1. 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 . - DOI
1. 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.
1. 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.

Publication types

Actions

MeSH terms

Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions

Substances

Actions
Actions

LinkOut - more resources

Full Text Sources
- Springer
Research Materials
- NCI CPTC Antibody Characterization Program
Miscellaneous
- NCI CPTAC Assay Portal

[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 . - DOI

[2] 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 . - DOI

[3] 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 . - DOI

[4] 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 . - DOI

[5] 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 . - DOI

[6] 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 . - DOI

[7] 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.

[8] 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.

[9] 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.

[10] 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.

Save citation to file

Email citation

Add to Collections

Add to My Bibliography

Your saved search

Create a file for external citation management software

Your RSS Feed

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

Affiliations

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

Authors

Affiliations

Abstract

Similar articles

References

Publication types

MeSH terms

Substances

LinkOut - more resources

Full Text Sources

Research Materials

Miscellaneous

Abstract

Similar articles

References

Publication types

MeSH terms

Substances

Related information

LinkOut - more resources

Full Text Sources

Research Materials

Miscellaneous