C3 glomerulonephritis

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C3 glomerulonephritis

ORPHA:329931Histopathological subtype

What it is

A histological subtype of C3 glomerulopathy characterized by C3 deposition in renal tissue in the absence or near-absence of immunoglobulin deposits, in a patient with the classic clinical features of glomerulonephritis and the electron microscopic findings of predominant subendothelial, occasionally subepithelial (so-called ''humps''), and intramembranous deposits, but without the typical electron-dense deposits of dense deposit disease.

Key facts

Inheritance
Autosomal dominant
Classified as
Histopathological subtype

Recorded for the broader condition

Prevalence
1-9 / 1 000 000 (annual incidence, Europe)C3 glomerulopathy
Age of onset
All agesC3 glomerulopathy

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

Genes

CFHDisease-causing germline mutation(s)
CFHR1Disease-causing germline mutation(s)
CFHR5Disease-causing germline mutation(s)
CFHR2Part of a fusion gene
CFHR3Part of a fusion gene

ICD-10 codes

N03.5filed under a broader ICD-10 category — shared with 2 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MONDO 0013892OMIM 609814OMIM 614809UMLS C4055342

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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