Immunotactoid or fibrillary glomerulopathy
Synonyms: Immunotactoid or fibrillary glomerulonephritis
- Type
- Clinical group
- Group
- Group of disorders
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Immunotactoid or fibrillary glomerulopathy is a group of very rare glomerular diseases, composed of immunotactoid glomerulopathy (ITG) and non-amyloid fibrillary glomerulopathy (non-amyloid FGP), that are characterized by mesangial deposition of monoclonal microtubular or polyclonal fibrillar deposits. Both present clinically with nephrotic range proteinuria, hematuria and renal insufficiency leading to renal failure in many cases. ITG is more likely to manifest with underlying lymphoproliferative disease, hypocomplementemia, dysproteinemia, monoclonal gammopathy or occult cryoglobulinemia. Non-amyloid FGP is 10 times more frequent than ITG.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Immunotactoid or fibrillary glomerulopathy | Immunotactoid or fibrillary glomerulonephritis |
| fr | Glomérulopathie immunotactoïde ou fibrillaire | Glomérulonéphrite immunotactoïde ou fibrillaire |
| de | Glomerulopathie, immunotaktoide oder fibrilläre | Glomerulonephritis, immunotaktoide oder fibrilläre |
| es | Glomerulopatía inmunotactoide o fibrilar | Glomerulonefritis fibrilar o inmunotactoide · Glomerulonefritis inmunotactoide o fibrilar |
| it | Glomerulopatia immunotattoide o fibrillare | |
| nl | Immunotactoïde of fibrillaire glomerulopathie | Immunotactoïde of fibrillaire glomerulonefritis |
| pt | Glomerulopatia imunotactóide ou fibrilar | Glomerulonefrite imunotactóide ou fibrilar |
| pl | Glomerulopatia immunotaktoidalna lub włókienkowa | = en |
| cs | Imunotaktoidní nebo fibrilární glomerulopatie | Imunotaktoidní nebo fibrilární glomerulonefritida |
| uk | Імунотактоїдна або фібрилярна гломерулопатія | Імунотактоїдний або фібрилярний гломерулонефрит |
| zh | 免疫触须样或纤维性肾小球肾病 | 免疫触须样或纤维样肾小球病 |
| tr | İmmünotaktoid veya fibriler glomerülopati | İmmünotaktoid veya fibriler glomerülonefrit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 0019605 | E | validated | |
| UMLS | C5680195 | E | validated | |
| GARD | 12741 | 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
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | <1 / 1 000 000 | — | Europe | Class only | Not yet validated | 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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/91137
- This record as JSON: /data/d/91137.json