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C3 glomerulopathy in cystic fibrosis: a case report

Domenico Santoro1, Rossella Siligato2, Carmela Vadalà2

  • 1Department of Clinical and Experimental Medicine, University of Messina, Via Faranda, 2-98123, Messina, Italy. dsantoro@unime.it.

BMC Nephrology
|March 30, 2018
PubMed

Insights

This study presents the first reported case of C3 glomerulonephritis (C3GN) in a patient with cystic fibrosis (CF). The findings highlight a potential link between CF-related chronic inflammation and complement system dysregulation in C3GN development.

Area of Science:

  • Nephrology
  • Immunology
  • Genetics

Background:

  • C3 glomerulonephritis (C3GN) is a rare kidney disease characterized by C3 complement protein deposition in the glomeruli.
  • It results from abnormal activation of the alternative complement pathway, often triggered by environmental factors.
  • Cystic fibrosis (CF) is a genetic disorder causing chronic inflammation and infection.

Observation:

  • A young woman with CF presented with persistent hematuria, proteinuria, and low C3 levels.
  • Renal biopsy confirmed C3GN, showing significant terminal complement pathway activation (sC5b-9 levels 1588 ng/ml).
  • Genetic analysis revealed known C3GN risk factors (CFH, THBD) and a novel C3 mutation (p.R102G) previously linked to age-related macular degeneration.

Findings:

  • This case represents the first documented instance of C3GN co-occurring with cystic fibrosis.
  • The patient exhibited genetic predispositions for C3GN and evidence of complement system dysregulation.
  • Treatment with ACE inhibitors stabilized kidney function.

Implications:

  • The co-existence of CF and C3GN offers a unique model to study chronic alternative pathway activation in inflammatory conditions.
  • Understanding this interplay may reveal new therapeutic targets for C3GN in patients with chronic inflammatory diseases.
  • Further research is warranted to explore the specific mechanisms linking CF and complement-mediated kidney damage.
Abstract

Keywords:
C3 glomerulopathyCystic fibrosisInflammationRenal biopsy

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