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Published on: July 8, 2020
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Crescentic IgA nephropathy in children.
Yuko Shima1, Koichi Nakanishi2, Taketsugu Hama1
1Department of Pediatrics, Wakayama Medical University, Wakayama City, Wakayama, Japan.
Pediatric Nephrology (Berlin, Germany)
|January 30, 2020
Summary
Crescentic IgA nephropathy (C-IgAN) in children is rare, often detected through school screenings. While treatment improved outcomes compared to prior studies, tubular atrophy indicates a poorer prognosis for C-IgAN patients.
Area of Science:
- Pediatric Nephrology
- Immunology
- Glomerular Diseases
Background:
- IgA nephropathy (IgAN) is a common primary glomerulonephritis.
- Crescentic IgA nephropathy (C-IgAN), defined by crescents in >50% of glomeruli, is a rare and severe form of IgAN.
- The clinical presentation and outcomes of C-IgAN in children are not well-characterized.
Purpose of the Study:
- To investigate the clinical and pathological characteristics of C-IgAN in children.
- To compare C-IgAN with non-C-IgAN in pediatric patients.
- To evaluate the renal survival and treatment outcomes in childhood C-IgAN.
Main Methods:
- Retrospective analysis of 515 children with biopsy-proven IgAN.
- Comparison of clinical and pathological findings between C-IgAN (n=25) and non-C-IgAN groups.
- Kaplan-Meier analysis for renal survival, with a mean follow-up of 6.0 years.
Main Results:
- C-IgAN accounted for 4.9% of childhood IgAN cases, with 64% detected via school screening.
- C-IgAN patients showed significantly higher gross hematuria, proteinuria, and acute glomerular lesions (M1, E1) but similar eGFR at diagnosis.
- Despite treatment, C-IgAN patients had significantly lower renal survival (77.1% vs. 92.6% at 13 years) and a higher rate of chronic renal failure (16%).
Conclusions:
- School screening is crucial for early detection of C-IgAN in children.
- Combination therapy with prednisolone and immunosuppressants improved renal outcomes compared to previous reports.
- The presence of tubular atrophy/interstitial fibrosis in C-IgAN is associated with a poorer long-term renal prognosis.
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