C3 glomerulopathy
Synonyms: Non-Ig-mediated MPGN · Non-Ig-mediated membranoproliferative glomerulonephritis · Non-immunoglobulin-mediated MPGN · Non-immunoglobulin-mediated membranoproliferative glomerulonephritis
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Multigenic/multifactorial
- Age of onset
- All ages
- Status
- active
A form of primary membranoproliferative glomerulonephritis characterized by the presence in renal biopsy samples of a glomerulonephritis with sole (or at least dominant) glomerular immunofluorescence staining for C3. Non-specific alterations or proliferative patterns with C3-dominant glomerular staining are also possible. Based upon electron microscopic findings, C3 glomerulopathy (C3G) may be further classified as C3 glomerulonephritis (C3GN) or Dense deposit disease (DDD).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | C3 glomerulopathy | Non-Ig-mediated MPGN · Non-Ig-mediated membranoproliferative glomerulonephritis · Non-immunoglobulin-mediated MPGN · Non-immunoglobulin-mediated membranoproliferative glomerulonephritis |
| fr | Glomérulopathie à dépôts de C3 | GNMP non médiée par les Ig · GNMP non médiée par les immunoglobulines · Glomérulonéphrite membranoproliférative non médiée par les Ig · Glomérulonéphrite membranoproliférative non médiée par les immunoglobulines · MPGN non médiée par les Ig · MPGN non médiée par les immunoglobulines |
| de | C3-Glomerulopathie | Glomerulonephritis, membranoproliferative, nicht Immunoglobulin-vermittelte |
| es | Glomerulopatía C3 | Glomerulonefritis membranoproliferativa no mediada por Ig · Glomerulonefritis membranoproliferativa no mediada por inmunoglobulinas · MPGN no mediada por Ig · MPGN no mediada por inmunoglobulinas |
| it | Glomerulopatia da C3 | Glomerulonefrite membranoproliferativa non-Ig-mediata · Glomerulopatia C3 · MPGN non mediata dalle immunoglobuline · MPGN non-Ig-mediata |
| nl | C3-glomerulopathie | Niet-Ig-gemedieerde MPGN · Niet-Ig-gemedieerde membranoproliferatieve glomerulonefritis · Niet-immunoglobuline-gemedieerde MPGN · Niet-immunoglobuline-gemedieerde membranoproliferatieve glomerulonefritis |
| pt | Glomerulopatia C3 | Glomerulonefrite membranoproliferativa não mediada por imunoglobulina · Glomerulonefrite membranoproliferativa não mediada por Ig |
| pl | Błoniasto-rozplemowe kłębuszkowe zapalenie nerek nie wywoływane przez immunoglobuliny | Błoniasto-rozplemowe kłębuszkowe zapalenie nerek nie wywoływane przez Ig · Glomerulopatia C3 · MPGN nie związane z Ig · MPGN nie związane z przeciwciałami MPGN |
| cs | Membranoproliferativní glomerulonefritida nezprostředkovaná imunoglobuliny | Glomerulopatie se sníženým C3 · MPGN nezprostředkovaná Ig · Membranoproliferativní glomerulonefritida nezprostředkovaná Ig · MPGN nezprostředkovaná imunoglobuliny |
| uk | С3 гломерулопатія | Не-Ig-опосередкований мембранопроліферативний гломерулонефрит · MPGN без Ig-опосередкування · Неімуноглобулін-опосередкований мембранопроліферативний гломерулонефрит · Неімуноглобулін-опосередкований MPGN |
| zh | C3肾小球病 | 非免疫球蛋白介导膜性肾小球肾炎 |
| tr | C3 glomerülopati | Ig-aracılı olmayan MPGN · İmmünoglobulin-aracılı olmayan MPGN · Ig-aracılı olmayan membranoproliferatif glomerülonefrit · İmmünoglobulin-aracılı olmayan membranoproliferatif glomerülonefrit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 609814 | BTNT | validated | |
| OMIM | 614809 | BTNT | validated | |
| ICD-10 | N03.5 | NTBT | attributed | validated |
| ICD-11 | MF8Y | NTBT | attributed | validated |
| MONDO | 0018013 | E | validated | |
| UMLS | C4087273 | E | validated | |
| MedDRA | 10077827 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:54370Primary membranoproliferative glomerulonephritis
rare genetic diseases; rare renal diseases; rare transplant-related disorders
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.15 / 100 000 | Europe | Value and class | Validated | 27056062[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
23 annotations · validated 2026-05-12| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0000790 | Hematuria | |
| Frequent (79-30%) | ||
| HP:0030888 | C3 nephritic factor positivity | |
| HP:0012622 | Chronic kidney disease | |
| HP:0005421 | Decreased circulating complement C3 concentration | |
| HP:0003259 | Elevated circulating creatinine concentration | |
| HP:0000822 | Hypertension | |
| HP:0000793 | Membranoproliferative glomerulonephritis | |
| HP:0012574 | Mesangial hypercellularity | |
| HP:0012593 | Nephrotic range proteinuria | |
| HP:0000093 | Proteinuria | |
| HP:0003774 | Stage 5 chronic kidney disease | |
| Occasional (29-5%) | ||
| HP:0030469 | Abnormal dark-adapted electroretinogram | |
| HP:0001919 | Acute kidney injury | |
| HP:0034001 | Anti-Complement factor H antibody positivity | |
| HP:0002960 | Autoimmunity | |
| HP:0008653 | Crescentic glomerulonephritis | |
| HP:0045042 | Decreased circulating complement C4 concentration | |
| HP:0011510 | Drusen | |
| HP:0009125 | Lipodystrophy | |
| HP:0000100 | Nephrotic syndrome | |
| HP:0031047 | Paraproteinemia | |
| HP:0000572 | Visual loss | |
| HP:0030506 | Yellow/white lesions of the retina | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 20301598[PMID]_30692664[PMID]_30077216[PMID]_38076230[PMID]_30692664[PMID]_32943008[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/329918
- This record as JSON: /data/d/329918.json