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KMID : 1040420210250010029
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Childhood Kidney Diseases
2021 Volume.25 No. 1 p.29 ~ p.34
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C3 Glomerulonephritis associated with Anti-complement Factor H Autoantibodies in an Adolescent Male: A Case Report
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Hyun Hye-Sun
Kang Hee-Gyung
Cho Ui-Ju
Ha Il-Soo
Cheong Hae-Il
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Abstract
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C3 glomerulonephritis (C3GN), a rare condition associated with dysregulation of the alternative pathway of the complement system, is histopathologically characterized by isolated or dominant C3 deposition in the renal glomeruli. We report a case of C3GN associated with anti-complement factor H (CFH) autoantibodies and CHF-related protein deficiency in an adolescent male. A 16-year-old adolescent male was admitted to a hospital with a 1-month history of generalized edema prior to presentation. Persistent microscopic hematuria and low serum C3 levels were incidentally detected at 7 and 10 years of age, respectively. Laboratory test results revealed hypoalbuminemia, nephrotic-range proteinuria, microscopic hematuria, and normal serum creatinine levels. The serum C3 and C4 levels were 17 mg/dL (normal 80?150 mg/dL) and 22 mg/mL (17?40 mg/mL), respectively. Renal biopsy showed typical features of C3GN. Further investigations revealed positive results on plasma anti-CFH autoantibody testing and a homozygous deletion of CFHR1 and CFHR3, which encode CFH-related proteins 1 and 3, respectively. Proteinuria persisted despite treatment with intravenous methylprednisolone, mycophenolate mofetil, and angiotensin-receptor blocker; however, his renal function remained stable. In conclusion, anti-CFH autoantibodies serve as important contributors to C3GN. This is the first case report that describes C3GN in an adolescent Korean male with anti-CFH autoantibodies and homozygous CFHR1 and CFHR3 deletion.
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KEYWORD
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C3 glomerulonephritis, Hypocomplementemia, Anti-complement factor H autoantibodies, Complement factor H related proteins
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