Fibroblastic rheumatism
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood
- Status
- active
A rare rheumatologic disease characterized by sudden onset of symmetric inflammatory distal polyarthritis and multiple firm cutaneous nodules with predilection for the upper and lower extremities. Patients often develop sclerodactyly and joint contractures. Skin biopsy shows fibroblastic proliferation in a matrix of thickened collagen fibers, with loss of elastic fibers and no mucin deposition.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Fibroblastic rheumatism | |
| fr | Rhumatisme fibroblastique | |
| de | Rheumatismus, fibroblastischer | |
| es | Reumatismo fibroblástico | |
| it | Reumatismo fibroblastico | |
| nl | Fibroblastische reuma | |
| pt | Reumatismo fibroblástico | |
| pl | Reumatyzm fibroblastyczny | |
| cs | Fibroblastický revmatizmus | |
| uk | Фібробластичний ревматизм | |
| zh | 成纤维细胞性风湿病 | |
| tr | Fibroblastik romatizma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M06.4 | NTBT | attributed | validated |
| ICD-11 | FB51.3 | E | specific | validated |
| MONDO | 0018783 | E | validated | |
| UMLS | C1302753 | 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:486955Rare pediatric rheumatologic disease
rare systemic or rheumatologic diseases of childhood - ORPHA:182231Rare rheumatologic disease
rare systemic and rheumatological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 30 cases | Worldwide | Case(s) | Validated | 25545215[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_25545215[PMID] |
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/477650
- This record as JSON: /data/d/477650.json