Primary membranous glomerulonephritis
Synonyms: Idiopathic membranous glomerulonephritis · Primary membranous nephropathy
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
A rare glomerular disease, histologically characterized by thickening of the capillary wall, with immune deposits predominantly containing IgG4 and C3 on the sub-epithelial side, and typically manifesting with nephrotic syndrome.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary membranous glomerulonephritis | Idiopathic membranous glomerulonephritis · Primary membranous nephropathy |
| fr | Glomérulonéphrite extra membraneuse primitive | Glomérulonéphrite extra-membraneuse idiopathique · Néphropathie extra-membraneuse primitive |
| de | Glomerulonephritis, primäre membranöse | Glomerulonephritis, idiopathische membranöse · Nephropathie, primäre membranöse |
| es | Glomerulonefritis membranosa primaria | Glomerulonefritis membranosa idiopática · Nefropatía membranosa primaria |
| it | Glomerulonefrite membranosa primitiva | |
| nl | Primaire membraneuze glomerulonefritis | Idiopathische membraneuze glomerulonefritis · Primaire membraneuze nefropathie |
| pt | Glomerulonefrite membranosa, idiopática | |
| pl | Idiopatyczne błoniaste kłębuszkowe zapalenie nerek | = en |
| cs | Idiopatická membranózní glomerulonefritida | = en |
| uk | Первинний мембранозний гломерулонефрит | Ідіопатичний мембранозний гломерулонефрит · Первинна мембранозна нефропатія |
| zh | 原发性膜性肾小球肾炎 | 特发性膜性肾小球肾炎 |
| tr | Primer membranöz glomerülonefrit | Primer membranöz nefropati · İdiyopatik membranöz glomerülonefrit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 614692 | E | validated | |
| ICD-10 | N05.2 | NTBT | attributed | validated |
| ICD-11 | GB40 | NTBT | attributed | validated |
| MONDO | 0013860 | E | validated | |
| UMLS | C0086445 | E | validated | |
| GARD | 9180 | 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:93548Glomerular disease
rare renal diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.9194 / 100 000 | Europe | Value and class | Validated | 21068142[PMID]_ORPHANET |
| Annual incidence | 1-9 / 1 000 000 | 0.8103 / 100 000 | Worldwide | Value and class | Validated | 21068142[PMID]_ORPHANET |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | 21068142[PMID]_34593809[PMID]_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.
HPO phenotypes
1 annotations · validated 2024-03-04| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0034002 | Anti-phospholipase A2 receptor antibody positivity | |
Annotation source: 34277215[PMID]_34169209[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/97560
- This record as JSON: /data/d/97560.json