Juvenile temporal arteritis
Synonyms: JTA · Non-giant cell granulomatous temporal arteritis with eosinophilia
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Childhood
- Status
- active
A rare vasculitis characterized by mostly unilateral inflammation of the temporal artery affecting patients up to fifty years of age. Patients typically present with a lump in the temporal region or a prominent temporal artery and often have headaches. Males are more frequently affected than females, and major systemic symptoms, ocular complications, and a biological inflammatory syndrome are usually absent, although peripheral blood eosinophilia may be seen. Histopathological analysis shows arteritis predominantly of the intima with possible extension to the perivascular tissue. The presence of granuloma, giant cells or fibrinoid necrosis is rare. Clinical relapses after one episode are uncommon.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Juvenile temporal arteritis | JTA · Non-giant cell granulomatous temporal arteritis with eosinophilia |
| fr | Artérite temporale juvénile | Artérite temporale granulomateuse éosinophilique sans cellules géantes |
| de | Arteriitis temporalis, juvenile | JTA · Temporalarteriitis, granulomatöse, mit Eosinophilie, nicht Riesenzelltyp |
| es | Arteritis temporal juvenil | Arteritis temporal granulomatosa de células no gigantes con eosinofilia · JTA |
| it | Arterite temporale giovanile | Arterite temporale granulomatosa non a cellule giganti con eosinofilia |
| nl | Juveniele arteriitis temporalis | JTA · Niet-reuscellige granulomateuze temporale arteriitis met eosinofilie |
| pt | Arterite temporal juvenil | Arterite temporal granulomatosa de células não-gigantes com eosinofilia |
| pl | Młodzieńcze zapalenie tętnicy skroniowej | Nie-olbrzymiokomórkowe ziarniniakowe zapalenie tetnicy skroniowej z eozynofilią |
| cs | Juvenilní temporální arteritida | Non-obrovskobuněčná granulomatózní temporální arteritida s eosinofilií |
| uk | Ювенільний скроневий артеріїт | JTA · Негігантоклітинний гранулематозний скроневий артеріїт з еозинофілією |
| zh | 巨细胞性动脉炎 | 非巨细胞肉芽肿性颞动脉炎伴嗜酸性粒细胞增多症 |
| tr | Jüvenil temporal arterit | JTA · Eozinofili ile seyreden dev hücreli olmayan granülomatöz temporal arterit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L95.8 | NTBT | attributed | validated |
| ICD-11 | 4A44.Y | NTBT | index | validated |
| MONDO | 0016848 | E | validated | |
| UMLS | C0751547 | E | validated | |
| GARD | 3068 | 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:156140Predominantly large-vessel vasculitis
rare systemic and rheumatological diseases; rare circulatory system 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 | 20 cases | Worldwide | Case(s) | Validated | ORPHANET | |
| Point prevalence | <1 / 1 000 000 | — | 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
8 annotations · validated 2016-07-29| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0002315 | Headache | |
| HP:0001974 | Increased total leukocyte count | |
| HP:0200036 | Skin nodule | |
| Occasional (29-5%) | ||
| HP:0003193 | Allergic rhinitis | |
| HP:0000509 | Conjunctivitis | |
| HP:0001880 | Increased total eosinophil count | |
| Excluded (0%) | ||
| HP:0002637 | Cerebral ischemia | |
| HP:0002633 | Vasculitis | |
Annotation source: 19748701[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/26137
- This record as JSON: /data/d/26137.json