Primary membranoproliferative glomerulonephritis
Synonyms: Mesangiocapillary glomerulonephritis · Primary MPGN
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare glomerular disease characterized by a pattern of glomerular injury on kidney biopsy with characteristic light microscopic changes: mesangial hypercellularity, endocapillary proliferation, and thickening of the glomerular basement membrane (GBM). On the basis of immunofluorescence (IF) the disorder is divided into C3 glomerulopathy (C3G) or immunoglobulin-mediated membranoproliferative glomerulonephritis. Through electron microscopy C3G is further divided into Dense deposit disease, with highly electrondense deposits in the glomerular basement membrane, and C3 glomerulonephritis, with mesangial, intramembranous, subendothelial and subepithelial deposits. Secondary causes (autoimmune, infectious, malignancies) are excluded.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary membranoproliferative glomerulonephritis | Mesangiocapillary glomerulonephritis · Primary MPGN |
| fr | Glomérulonéphrite membranoproliférative primaire | Glomérulonéphrite mésangiocapillaire · MPGN primaire |
| de | Glomerulonephritis, membranoproliferative | Glomerulonephritis, mesangiokapilläre · MPGN, primär |
| es | Glomerulonefritis membranoproliferativa primaria | GNMP primaria · Glomerulonefritis mesangiocapilar |
| it | Glomerulonefrite membranoproliferativa primitiva | Glomerulonefrite mesangio-capillare · MPGN |
| nl | Primaire membranoproliferatieve glomerulonefritis | Mesangiocapillaire glomerulonefritis · Primaire MPGN |
| pt | Glomerulonefrite membrano-proliferativa primária | Glomerulonefrite mesangiocapilar · MPGN primária |
| pl | Pierwotne błoniasto-rozplemowe kłębuszkowe zapalenie nerek | Błoniasto-rozplemowe kłębuszkowe zapalenie nerek · MPGN |
| cs | Primární membranoproliferativní glomerulonefritida | MPGN · Mesangiokapilární glomerulonefritida |
| uk | Первинний мембранопроліферативний гломерулонефрит | Мезангіокапілярний гломерулонефрит · Первинна MPGN |
| zh | 原发性膜增生性肾小球肾炎 | 系膜毛细血管性肾小球肾炎 |
| tr | Primer membranoproliferatif glomerülonefrit | Primer MPGN · Mezanjiyokapiller glomerülonefrit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 305800 | BTNT | validated | |
| OMIM | 609814 | BTNT | validated | |
| OMIM | 614809 | BTNT | validated | |
| OMIM | 615008 | BTNT | validated | |
| ICD-10 | N05.5 | NTBT | attributed | validated |
| ICD-11 | MF8Y | NTBT | index | validated |
| MONDO | 0018904 | E | validated | |
| MeSH | D015432 | E | validated | |
| UMLS | C0017662 | E | validated | |
| GARD | 11982 | E | validated | |
| MedDRA | 10018370 | 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 (2):
- ORPHA:183586Genetic glomerular disease
rare genetic diseases - ORPHA:93548Glomerular disease
rare renal diseases; rare transplant-related disorders
Genes
0 direct · 1 via subtypesOrphanet records no gene directly on this code.
Recorded by Orphanet on subtypes of this disorder:
| Gene | Subtype | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|---|
| DGKE | ORPHA:329903Immunoglobulin-mediated membranoproliferative glomerulonephritis | Disease-causing germline mutation(s) in | Assessed | HGNC:2852 | ENSG00000153933 | 601440 | 17q22 | diacylglycerol kinase epsilon | 23274426[PMID] |
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | 16 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2012[OTHER] |
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
16 annotations · validated 2019-05-27| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0000793 | Membranoproliferative glomerulonephritis | |
| Frequent (79-30%) | ||
| HP:0030888 | C3 nephritic factor positivity | |
| HP:0012622 | Chronic kidney disease | |
| HP:0005421 | Decreased circulating complement C3 concentration | |
| HP:0004746 | Glomerular subendothelial electron-dense deposits | |
| HP:0000822 | Hypertension | |
| HP:0002907 | Microscopic hematuria | |
| HP:0000100 | Nephrotic syndrome | |
| HP:0000093 | Proteinuria | |
| HP:0000083 | Renal insufficiency | |
| Occasional (29-5%) | ||
| HP:0001919 | Acute kidney injury | |
| HP:0003073 | Hypoalbuminemia | |
| HP:0003774 | Stage 5 chronic kidney disease | |
| Very rare (<4-1%) | ||
| HP:0001977 | Abnormal thrombosis | |
| HP:0011510 | Drusen | |
| HP:0001658 | Myocardial infarction | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 27056062[PMID]_22798508[PMID]_22802081[PMID]_30854618[PMID]_26972096[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/54370
- This record as JSON: /data/d/54370.json