ORPHA:329903
Immunoglobulin-mediated membranoproliferative glomerulonephritis
Synonyms: Ig-mediated MPGN · Ig-mediated membranoproliferative glomerulonephritis · Immunoglobulin-mediated MPGN
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Multigenic/multifactorial, Unknown
- Status
- active
A primary form of membranoproliferative glomerulonephritis (MPGN) characterized by deposition in the renal glomeruli of immunoglobulin with complement fractions, especially C3. Clinical presentation may range from nephrotic syndrome and acute kidney injury to asymptomatic proteinuria and hematuria.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Immunoglobulin-mediated membranoproliferative glomerulonephritis | Ig-mediated MPGN · Ig-mediated membranoproliferative glomerulonephritis · Immunoglobulin-mediated MPGN |
| fr | Glomérulonéphrite membranoproliférative médiée par les immunoglobulines | GNMP médiée par les Ig · GNMP médiée par les immunoglobulines · Glomérulonéphrite membranoproliférative médiée par les Ig · MPGN médiée par les Ig · MPGN médiée par les immunoglobulines |
| de | Glomerulonephritis, membranoproliferative, Immunoglobulin-vermittelte | Ig-mediated membranoproliferative Glomerulonephritis · Ig-vermittelte MPGN · Immunoglobulin-vermittelte MPGN |
| es | Glomerulonefritis membranoproliferativa mediada por inmunoglobulinas | Glomerulonefritis membranoproliferativa mediada por Ig · MPGN mediada por Ig · MPGN mediada por inmunoglobulinas |
| it | Glomerulonefrite membranoproliferativa mediata dalle immunoglobuline | Glomerulonefrite membranoproliferativa Ig-mediata · MPGN Ig-mediata · MPGN mediata dalle immunoglobuline |
| nl | Immunoglobuline-gemedieerde membranoproliferatieve glomerulonefritis | Ig-gemedieerde MPGN · Ig-gemedieerde membranoproliferatieve glomerulonefritis · Immunoglobuline-gemedieerde MPGN |
| pt | Glumerulonefrite membranosa proliferativa associada a imunoglobulina | |
| pl | Błoniasto-rozplemowe kłębuszkowe zapalenie nerek wywoływane przez immunoglobuliny | Błoniasto-rozplemowe kłębuszkowe zapalenie nerek wywoływane przez Ig · MPGN wywołane przez Ig · MPGN wywołane przez immunoglobuliny |
| cs | Membranoproliferativní glomerulonefritida zprostředkovaná imunoglobuliny | MPGN zprostředkovaná Ig · Membranoproliferativní glomerulonefritida zprostředkovaná Ig · MPGN zprostředkovaná imunoglobuliny |
| uk | Імуноглобулін-опосередкований мембранопроліферативний гломерулонефрит | Ig-опосередкований мембранопроліферативний гломерулонефрит · Ig-опосередкований MPGN · Імуноглобулін-опосередкований MPGN |
| zh | 免疫球蛋白介导膜性肾小球肾炎 | Ig介导膜性肾小球肾炎 |
| tr | İmmünoglobulin aracılı membranoproliferatif glomerülonefrit | Ig-aracılı MPGN · İmmünoglobulin-aracılı MPGN · Ig-aracılı membranoproliferatif glomerülonefrit |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 615008 | E | validated | |
| ICD-10 | N03.5 | NTBT | attributed | validated |
| MONDO | 0014005 | E | validated | |
| UMLS | C5679858 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:54370Primary membranoproliferative glomerulonephritis
rare genetic diseases; rare renal diseases; rare transplant-related disorders
No children.
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Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| DGKE | Disease-causing germline mutation(s) in | Assessed | HGNC:2852 | ENSG00000153933 | 601440 | 17q22 | diacylglycerol kinase epsilon | 23274426[PMID] |
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Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
13 annotations · validated 2026-05-18| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0012574 | Mesangial hypercellularity | |
| Frequent (79-30%) | ||
| HP:0030888 | C3 nephritic factor positivity | |
| HP:0005421 | Decreased circulating complement C3 concentration | |
| HP:0000969 | Edema | |
| HP:0000790 | Hematuria | |
| HP:0000822 | Hypertension | |
| HP:0012593 | Nephrotic range proteinuria | |
| HP:0000093 | Proteinuria | |
| HP:0000092 | Renal tubular atrophy | |
| HP:0033495 | Segmental glomerulosclerosis | |
| Occasional (29-5%) | ||
| HP:0034001 | Anti-Complement factor H antibody positivity | |
| HP:0033319 | Fibrous crescent | |
| HP:0000083 | Renal insufficiency | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 35777834[PMID]_35777835[PMID]_42037281[PMID]_32622830[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/329903
- This record as JSON: /data/d/329903.json