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Orphanet Rare Disease Ontology
| Preferred Name | Juvenile temporal arteritis | |
| Synonyms |
Non-giant cell granulomatous temporal arteritis with eosinophilia JTA |
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| Definitions |
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. |
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| ID |
http://www.orpha.net/ORDO/Orphanet_26137 |
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| alternative_term |
Non-giant cell granulomatous temporal arteritis with eosinophilia JTA
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|
| definition |
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.
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| definition_citation |
Orphanet
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| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=26137 |
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| has_age_of_onset |
Childhood
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| has_inheritance |
Unknown
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| hasDbXref |
UMLS:C0751547 ICD-10:L95.8 ICD-11:4A44.Y
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| label |
Juvenile temporal arteritis
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| notation |
ORPHA:26137
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| part_of | ||
| prefixIRI |
ORDO:Orphanet_26137
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| prefLabel |
Juvenile temporal arteritis
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| present_in |
Worldwide AND has_cases/families_value : 20.0 (Case) Worldwide AND has_point_prevalence_range : <1 / 1 000 000
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| treeView | ||
| subClassOf |
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