C3 Glomerulonephritis associated with Anti-complement Factor H Autoantibodies in an Adolescent Male: A Case Report
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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.
Abstract
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.
참고문헌 (15)
- Caravaca-Fontán F, "Update on C3glomerulopathy : a complement-mediated disease", Nephron, 144, pp.272~280 (2020)
- Cook HT, "Histopathology of MPGN and C3 glomerulopathies", Nat Rev Nephrol, 11, pp.14~22 (2015)
- Ahmad SB, "C3 glomerulopathy : pathogenesis and treatment", Adv Chronic Kidney Dis, 27, pp.104~110 (2020)
- Dragon-Durey MA, "Anti-factor H autoantibodies associated with atypical hemolytic uremic syndrome", J Am Soc Nephrol, 16, pp.555~553 (2005)
- Dragon-Durey MA, "Clinical features of anti-factor H autoantibodyassociated hemolytic uremic syndrome", J Am Soc Nephrol, 21, pp.2180~2187 (2010)
- Lee BH, "Atypical hemolytic uremic syndrome associated with complement factor H autoantibodies and CFHR1", CFHR3 deficiency, 2009, pp.66, 336~340
- Lee JM, "Atypical hemolytic uremic syndrome : Korean pediatric series", Pediatr Int, 57, pp.431~438 (2015)
- Blanc C, "Anti-factor H autoantibodies in C3 glomerulopathies and in atypical hemolytic uremic syndrome: one target, two diseases", J Immunol, 194, pp.5129~5138 (2015)
- Durey MA, "Anti-complement-factor H-associated glomerulopathies", Nat Rev Nephrol, 12, pp.563~578 (2016)
- Salvadori M, "Reclassification of membranoproliferative glomerulonephritis : identification of a new GN : C3GN", World J Nephrol, 5, pp.308~320 (2016)
- Sethi S, "Mayo Clinic", Renal Pathology Society Consensus Report on Pathologic Classification, Diagnosis, and Reporting of GN, 2016, pp.27, 1278~1287
- Servais A, "Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3glomerulopathies", Kidney Int, 82, pp.454~464 (2012)
- Bomback AS, "C3 glomerulonephritis and dense deposit disease share a similar disease course in a large United States cohort of patients with C3 glomerulopathy", Kidney Int, 93, pp.977~985 (2018)
- Ravindran A, "C3glomerulopathy : ten years' experience at Mayo Clinic", Mayo Clin Proc, 93, pp.991~1008 (2018)
- Riedl M, "C3 glomerulopathy", Pediatr Nephrol, 32, pp.43~57 (2017)