Lupus nephritis (LN) is a common and serious complication of pediatric-onset systemic lupus erythematosus (pSLE) that can cause long-term glucocorticoid/immune suppressant use and even end-stage renal disease. Interstitial inflammation (II) is a pathology component in LN that is an early predictor for poor renal outcomes, particularly when it presents with tubulointerstitial components. However, II has shown inconsistent findings in relation to ESRD in previous studies, and limited sampling from renal tissue may distort the II score and chronicity. Therefore, a more detailed study in renal pathology is required, which is what the present study focused on.
The study analyzed the risk of ESRD based on different II scores in 48 pSLE patients with class III/IV LN, and studied 3D renal pathology and immunofluorescence (IF) staining of CD3, 19, 20, and 138 in patients with high II scores but low chronicity. Results showed that pSLE LN patients with II scores of 2 or 3 had a higher risk of ESRD than those with II scores of 0 or 1. The study also revealed a more complex inflammation in situ in LN, such as closely packed T:B aggregates and high interstitial B cell densities being associated with protection of renal survival, while high CD4-CD8- T cell densities were associated with acute and chronic renal failure.
In conclusion, this study provides unique data on LN, including 3D pathology and different in situ Syndecan-1 patterns in LN patients. It highlights the importance of II as an early predictor for poor renal outcomes in pSLE LN patients and the complexity of the inflammation in situ in LN. The study also suggests that a more detailed study in renal pathology is required to improve the understanding of the pathogenesis of LN and improve its diagnosis and management.
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Sources: Huang, Yung-Chieh, et al. “The Role of New 3D Pathology and Lymphocyte Expression of Interstitial Inflammation in Pediatric-Onset Lupus Nephritis.” International Journal of Molecular Sciences 24.4 (2023): 3512.