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C4 Glomerulopathy: A Disease Entity Associated With C4d Deposition
Sanjeev Sethi1, Patrick S Quint1, Conall M O'Seaghdha2
1Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.
Summary
A new kidney disease, C4 glomerulopathy, shows bright C4d staining with minimal complement or immunoglobulin deposits. This finding aids in diagnosing specific complement-mediated glomerulonephritis.
Area of Science:
- Nephrology
- Immunology
- Pathology
Background:
- Complement-mediated glomerulonephritis typically shows dominant C3 staining.
- Current diagnostic criteria rely on C3 and immunoglobulin deposits.
Observation:
- A novel entity, C4 glomerulopathy, is characterized by bright C4d staining.
- This condition presents with minimal or absent C3 and immunoglobulin deposits.
- C4 glomerulopathy includes C4 dense deposit disease and C4 glomerulonephritis.
Findings:
- Three patients presented with proteinuria and hematuria, with varying kidney function.
- Kidney biopsies revealed glomerulonephritis with prominent C4d staining.
- Mass spectrometry confirmed C4 presence, localized to the C4d portion.
Implications:
- C4 glomerulopathy represents a distinct form of complement-mediated kidney disease.
- This discovery expands the understanding of complement's role in glomerular injury.
- Further research is needed to elucidate the etiology of C4 glomerulopathy.
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