Nephrogenic systemic fibrosis
Synonyms: Nephrogenic fibrosing dermopathy
- Type
- Disease
- Group
- Disorder
- Status
- active
Nephrogenic systemic fibrosis (NSF) is a rare systemic fibrosing condition observed in renally impaired patients and characterized by a hardening and thickening of the skin with fibrotic plaques or papules, pruritus, joint pain and stiffness, muscle weakness, limitation of range of motion, and yellowed eyes. It is generally associated with administration of gadolinium-based magnetic resonance imaging contrast agents (GBCA) in patients with kidney disease.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Nephrogenic systemic fibrosis | Nephrogenic fibrosing dermopathy |
| fr | Fibrose systémique fibrogénique | Dermopathie fibrosante néphrogénique |
| de | Nephrogene systemische Fibrose | NFS · Nephrogene fibrosierende Dermopathie |
| es | Fibrosis sistémica nefrogénica | Dermopatía fibrosante nefrogénica |
| it | Fibrosi sistemica nefrogenica | Dermopatia fibrosante nefrogenica |
| nl | Nefrogene systemische fibrose | Nefrogene fibroserende dermopathie |
| pt | Dermopatia fibrosante nefrogénica | |
| pl | Nefrogenne włóknienie układowe | Nefrogenna dermopatia włóknista |
| cs | Nefrogenní systémová fibróza | Nefrogenní fibrotizující dermopatie · NSF · NFD |
| uk | Нефрогенний системний фіброз | Нефрогенна фіброзуюча дермопатія |
| zh | 肾源性系统性纤维化 | 肾源性纤维性皮肤病 |
| tr | Nefrojenik sistemik fibroz | Nefrojenik fibrozan dermopati |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M72.8 | NTBT | attributed | validated |
| ICD-11 | FB51.Y | NTBT | index | validated |
| MONDO | 0015294 | E | validated | |
| UMLS | C3888044 | E | validated | |
| GARD | 9725 | E | validated | |
| MedDRA | 10067467 | 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:90077Other acquired skin disease
rare skin diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/137617
- This record as JSON: /data/d/137617.json