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Clinical, laboratory, and pathological correlates of crescent formation in biopsy-proven pediatric IgA vasculitis
Jixiang Xu1,2, Jiaqi Di3, Xia Zhang4,5
1Department of Pediatrics, The First Affiliated Hospital, Henan University of Chinese Medicine, No. 19 Renmin Road, Zhengzhou, 450003, Henan, China.
Insights
Crescent formation in pediatric IgA vasculitis nephritis (IgAVN) indicates a more severe disease. Kidney biopsy is crucial for assessing crescents and related kidney lesions.
Area of Science:
- Pediatric Nephrology
- Renal Pathology
Background:
- Crescent formation is frequent in pediatric IgA vasculitis nephritis (IgAVN).
- Clinicopathological correlates and significance of crescent burden and activity in IgAVN are not well understood.
Purpose of the Study:
- To investigate the clinicopathological features associated with crescent formation in pediatric IgAVN.
- To evaluate the significance of crescent burden and activity in IgAVN.
Main Methods:
- Retrospective analysis of 1,280 children with biopsy-proven IgAVN.
- Classification based on crescent presence, burden, and activity.
- Logistic regression and sensitivity analyses were performed.
Main Results:
- Crescent formation occurred in 70.5% of children.
- Children with crescents presented with more severe clinical and pathological findings.
- Glomerulosclerosis, tubulointerstitial fibrosis/inflammation, endocapillary hypercellularity, and necrotizing lesions were linked to crescents.
Conclusions:
- Crescent formation in pediatric IgAVN signifies a severe clinicopathological phenotype.
- Coexisting active and chronic kidney lesions are important context for crescent interpretation.
- Kidney biopsy is essential for diagnosing crescent-related lesions in IgAVN.
Background:
Crescent formation is common in pediatric IgA vasculitis nephritis (IgAVN), but its clinicopathological correlates and the significance of crescent burden and activity remain unclear.
Methods:
We retrospectively analyzed 1,280 children with biopsy-proven IgAVN and available crescent assessment who were hospitalized at the Department of Pediatric Nephrology, The First Affiliated Hospital of Henan University of Chinese Medicine, between January 2013 and January 2021. Patients were classified by the presence or absence of crescents on kidney biopsy. Univariable and sequential multivariable logistic regression analyses were performed, with additional stratified, post hoc, and sensitivity analyses for crescent burden and active crescents.
Results:
Crescent formation was identified in 903/1,280 children (70.5%). Compared with those without crescents, children with crescents were older and more likely to have gross hematuria, gross gastrointestinal bleeding, at least one non-cutaneous extrarenal manifestation, and severe renal clinical involvement. They also had higher 24-h urinary protein excretion and D-dimer levels and more frequent active and chronic pathological lesions. In the fully adjusted model, glomerulosclerosis, tubulointerstitial fibrosis, tubulointerstitial inflammation, endocapillary hypercellularity, and glomerular necrotizing lesions remained independently associated with crescent formation. Higher crescent burden was associated with heavier proteinuria, higher D-dimer levels and more frequent active and chronic pathological lesions. In sensitivity analyses, tubulointerstitial inflammation and glomerular necrotizing lesions remained consistently associated across alternative crescent-related outcomes.
Conclusions:
In children with IgAVN, crescent formation is associated with a more severe clinicopathological phenotype and should be interpreted in the context of coexisting active and chronic kidney lesions. Kidney biopsy remains essential for defining crescent-related lesions.
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