Idiopathic non-lupus full-house nephropathy
Synonyms: Idiopathic non-lupus FHN
- Type
- Clinical syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare idiopathic glomerular clinical syndrome characterized by diffuse renal lesions that are indistinguishable from the lesions observed in systemic lupus erythematosus (SLE) in the absence of circulating autoantibodies and other systemic features necessary to meet the classification criteria for SLE. Patients may present with nephrotic syndrome, abnormal urinary sediment, acute renal insufficiency, progressive glomerulonephritis, and hypertension. Some patients have been reported to develop a progression to SLE over time.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic non-lupus full-house nephropathy | Idiopathic non-lupus FHN |
| fr | Néphropathie full-house non lupique idiopathique | FHN non lupique idiopathique · FHN séronégative idiopathique |
| de | Idiopathische non-Lupus Full-House-Nephropathie | Idiopathische non-Lupus FHN |
| es | Nefropatía full house no lúpica idiopática | FHN no lúpica idiopática |
| it | Nefropatia non lupica idiopatica ''full house'' | FHN non lupica idiopatica |
| nl | Idiopathische niet-lupus 'full house'-nefropathie | Idiopathische niet-lupus FHN |
| pt | Nefropatia não-lúpica full-house idiopática | |
| pl | Idiopatyczna nefropatia pełnoobjawowa nie związana z toczniem | = en |
| cs | Idiopatická nelupusová full-house nefropatie | = en |
| uk | Ідіопатична не вовчакова повна нефропатія | Ідіопатичний не вовчаковий FHN |
| zh | 特发性非狼疮性满堂亮肾病 | = en |
| tr | İdiyopatik lupus dışı full-house nefropati | İdiyopatik lupus olmayan FHN |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | N05 | NTBT | attributed | validated |
| ICD-11 | GB4Y | NTBT | attributed | validated |
| MONDO | 0035763 | E | validated | |
| UMLS | C5680132 | 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
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | 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
22 annotations · validated 2021-06-16| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0000093 | Proteinuria | |
| Frequent (79-30%) | ||
| HP:0004431 | Complement deficiency | |
| HP:0005421 | Decreased circulating complement C3 concentration | |
| HP:0045042 | Decreased circulating complement C4 concentration | |
| HP:0000822 | Hypertension | |
| HP:0000100 | Nephrotic syndrome | |
| HP:0000083 | Renal insufficiency | |
| Occasional (29-5%) | ||
| HP:0001919 | Acute kidney injury | |
| HP:0001369 | Arthritis | |
| HP:0003259 | Elevated circulating creatinine concentration | |
| HP:0012576 | Glomerular C3 deposition | |
| HP:0000099 | Glomerulonephritis | |
| HP:0001966 | Mesangial abnormality | |
| HP:0002907 | Microscopic hematuria | |
| HP:0000155 | Oral ulcer | |
| HP:0045073 | Serositis | |
| HP:0000988 | Skin rash | |
| HP:0100769 | Synovitis | |
| Very rare (<4-1%) | ||
| HP:0002715 | Abnormality of the immune system | |
| HP:0000707 | Abnormality of the nervous system | |
| Excluded (0%) | ||
| HP:0020151 | Anti-dsDNA antibody positivity | |
| HP:0002960 | Autoimmunity | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 28340077[PMID]_27270291[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/567544
- This record as JSON: /data/d/567544.json