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
. 2023 Aug 24;23(1):423.
doi: 10.1186/s12887-023-04243-3.

Comparative study on clinicopathological features and prognosis of IgA vasculitis nephritis and IgA nephropathy in children

Yan Lv  1   2 , Rui Fu  1 , Xiao-Jie Peng  3 , Ying Wang  2 , Ting-Ting Yin  2 , Yan-Qing Deng  2
Affiliations

Comparative study on clinicopathological features and prognosis of IgA vasculitis nephritis and IgA nephropathy in children

Yan Lv et al. BMC Pediatr. .

Abstract

Background: IgA vasculitis nephritis (IgAVN) and IgA nephropathy (IgAN) share several clinical and pathological characteristics, though distinctions also exist. Their interrelation, however, remains undefined. This study investigates the clinicopathological divergences and prognostic disparities in pediatric patients with IgAVN and IgAN.

Methods: Our study encompasses 809 pediatric patients with IgAVN and 236 with IgAN, all of whom underwent kidney biopsy. We utilized the Semiquantitative Classification (SQC) scoring system to juxtapose the pathologies of the two conditions, and performed a COX regression analysis to examine factors influencing their prognoses.

Results: Both patient groups demonstrated a predominance of males. A seasonality was observed, with a higher incidence of IgAN in the summer, and IgAVN in the fall (P < 0.0001). Patients with IgAN exhibited more severe tubulointerstitial injury, higher chronicity index, and total biopsy scores compared to those with IgAVN (P < 0.0001). Mesangial deposition intensity of complement C3, and the rate of pure IgA deposition, were found to be greater in patients with IgAVN compared to those with IgAN (P < 0.0001). The intensity of IgA deposition was also significantly higher in IgAVN patients (P = 0.003). IgAVN demonstrated a superior prognosis, with a higher rate of kidney remission (P < 0.0001). COX regression analysis indicated that interstitial fibrosis, as identified in the SQC pathology system, was associated with the prognosis of both conditions. Furthermore, the findings suggest that IgA deposition levels (IgA + + and IgA + + +) could potentially influence the prognosis of IgAVN.

Conclusions: Compared to IgAVN, IgAN manifests more severely with regard to renal impairment, interstitial damage, and prognosis. The disparities in immune complex deposition levels and locations within the kidneys support the hypothesis of IgAVN and IgAN as distinct diseases. Interstitial fibrosis may serve as a key pathological indicator within the SQC system associated with kidney prognosis in children with IgAVN and IgAN. The degree of IgA deposition could also be linked with the prognosis of IgAVN.

Keywords: Children; IgA nephropathy; IgA vasculitis nephritis; Kidney prognosis; SQC.

PubMed Disclaimer

Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Flow chart of case screening in IgA vasculitis nephritis (IgAVN) and IgA nephropathy (IgAN) groups
Fig. 2
Fig. 2
Kaplan–Meier analysis of kidney remission rates in IgA vasculitis nephritis (IgAVN) and IgA nephropathy (IgAN) (Log-rank test, P = 0.017)
Fig. 3
Fig. 3
Kaplan–Meier analysis of the effect of IgA deposition intensity on the kidney remission rates of IgA vasculitis nephritis (IgAVN) (Log-rank test, P < 0.0001)
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Zhang X, Xie X, Shi S, Liu L, Lv J, Zhang H. Plasma galactose-deficient immunoglobulin A1 and loss of kidney function in patients with immunoglobulin A vasculitis nephritis. Nephrol Dial Transplant. 2020;35(12):2117–2123. doi: 10.1093/ndt/gfz151. - DOI - PubMed
    1. Li X, Tang M, Yao X, Zhang N, Fan J, Zhou N, et al. A clinicopathological comparison between IgA nephropathy and Henoch-Schönlein purpura nephritis in children: use of the Oxford classification. Clin Exp Nephrol. 2019;23(12):1382–1390. doi: 10.1007/s10157-019-01777-8. - DOI - PubMed
    1. Working Group of the International IgA Nephropathy Network and the Renal Pathology Society; Cattran DC, Coppo R, Cook HT, Feehally J, Roberts IS, et al. The Oxford classification of IgA nephropathy: rationale, clinicopathological correlations, and classification. Kidney Int. 2009;76(5):534–545. - PubMed
    1. Working Group of the International IgA Nephropathy Network and the Renal Pathology Society; Roberts IS, Cook HT, Troyanov S, Alpers CE, Amore A, et al. The Oxford classification of IgA nephropathy: pathology definitions, correlations, and reproducibility. Kidney Int. 2009;76(5):546–556. - PubMed
    1. Trimarchi H, Barratt J, Cattran DC, Cook HT, Coppo R, Haas M, et al. Oxford Classification of IgA nephropathy 2016: an update from the IgA Nephropathy Classification Working Group. Kidney Int. 2017;91(5):1014–1021. doi: 10.1016/j.kint.2017.02.003. - DOI - PubMed

Publication types

Substances

LinkOut - more resources