Child Kidney Dis.  2025 Feb;29(1):24-31. 10.3339/ckd.25.001.

Clinicopathological differences in the activation pattern of the complement system between pediatric and adult lupus nephritis: a single centered retrospective study in Korea

• Park MJ
• Han MH
• Kim Ms
• Kim YJ
• Lee SJ
• Kim D
• Baek HS
• Cho MH

• KMID: 2565478
• DOI: http://doi.org/10.3339/ckd.25.001

Abstract

Purpose
Lupus nephritis (LN) can be caused by the complement activation. This study aimed to investigate the differences and clinical implications of the activation pattern of the complement system for pediatric and adult LN patients.
Methods
We retrospectively reviewed the medical records of 40 patients (14 pediatric and 26 adult patients) diagnosed with LN through kidney biopsy.
Results
The mean ages at diagnosis of pediatric and adult patients were 11.7±2.92 and 37.3±13.5 years, respectively. At the first LN diagnosis, compared with adult patients, pediatric patients had a higher estimated glomerular filtration rate and milder proteinuria; however, there was no statistical significance. The age-adjusted mean serum complement 3 value was significantly lower in the pediatric group (33.0±11.3 mg/dL) than in the adult group (50.8±25.2 mg/dL) (P<0.01). Based on the findings of kidney biopsy, no significant differences were observed in the severity of pathologic classification and the positive rate of complements between adults and children. However, the chronicity index score of adult patients was significantly higher than that of pediatric patients and in the case of complement 4d, despite a similar positive rate, the intensity was significantly stronger for adults (2.35±0.83 vs. 1.54±0.52, (P=0.04).
Conclusions
The activation pattern of the complement system in LN differs clinicopathologically between pediatric and adult patients and these differences might play an important role in the age-dependent prognosis of LN.

Keyword

Adult; Child; Complement; Lupus nephritis

Figure

• 

  Fig. 1. Representative biopsies showing the detection of C3 and C1q by immunofluorescence staining (magnification, ×400) and C4d by immunohistochemistry staining (magnification, ×400). Kidney biopsy staining results for C4d, C1q, and C3 were scored as 0, 1, 2, or 3 (0, absence of complement staining; 1, weak staining; 2, moderate staining; 3, strong staining). The histopathological features of all specimens were evaluated by two pathologists. C3, complement 3; C4d, complement 4d; C1q, complement 1q.

• 

  Fig. 2. Comparison of intensity of C4d staining between pediatric and adult patients with severe LN (ISN/RPS class IV). Periodic acid-Schiff (PAS) and immunohistochemical (IHC) stain for C4d in a biopsied kidney tissue of a 14-year-old girl with LN class IV (A), an 11-year-old girl with LN class IV (B), and PAS and IHC stain for C4d in biopsied kidney tissues of two adult patients with LN class IV (C, D). The intensity of IHC stain for C4d in adult patients with LN class IV is significantly stronger than that in pediatric patients with LN class IV (magnification, ×400). C4d, complement 4d; LN, lupus nephritis; ISN/RPS, International Society of Nephrology and Renal Pathology Society.

Reference

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