Giant cell arteritis
Definicija
Retki vaskulitis velikih krvnih sudova (LVV) karakteriše se upalom koja uglavnom pogađa arterije koje izviru iz luka aorte i ekstrakranijalnih grana karotidnih arterija. Kliničke manifestacije variraju, pri čemu dominira kranijalni fenotip sa simptomima poput glavobolje, klaudikacije vilice, osetljivosti vlasišta i vizuelnih simptoma, dok LVV fenotip obuhvata konstitucionalne simptome, reumatsku polimijalgiju i povremenu ishemiju ekstremiteta. Preklapanje između ova dva fenotipa je često.

Pretraga
Pun naziv
Giant cell arteritis
Kratki naziv
-
Sinonimi
Hortonova bolest
Orpha broj
397
Kategorija
Podkategorija
Naziv na stranom jeziku
-
Prevalenca
1-5 / 10 000
Nasleđivanje
Multigenic/multifactorial
Period početka bolesti
-
ICD 10
M31.6
OMIM
187360
UMLS
C0039483 C1956391
GARD
9615
MEDDRA
10018250 10043207
Tekstualni opis
GCA often starts insidiously with general symptoms, cranial manifestations (headache, jaw claudication, scalp tenderness, visual loss), and, in about 50% of patients, polymyalgia rheumatica. Visual symptoms due to an ischemic optic neuropathy occur in 20-30% of patients, and can rapidly lead to irreversible monocular blindness. Large artery disease, including carotid or subclavian arteries, may also occur. Thoracic aortitis with aneurysms occur in approximately 15% of patients but are generally a late complication of GCA.
Etiologija
The etiology of giant cell arteritis is unknown. Studies have linked genetic factors, infectious agents and a prior history of cardiovascular disease to the development of giant cell arteritis.
Prognoza
bolest_prognoza
Diferencijalna dijagnoza
In elderly patients presenting with general symptoms and elevated inflammatory markers, diagnoses of cancer or infection need to be considered. Symptoms of polymyalgia rheumatica can also point towards diagnoses of isolated polymyalgia rheumatica or rheumatoid arthritis. In some instances, biopsy-proven involvement of the temporal arteries can be seen in other systemic vasculitides, e.g., polyarteritis nodosa or microscopic polyangiitis.
Tretman
Glucocorticoids are highly and rapidly effective in GCA but are often associated with substantial morbidity in an elderly population. Adjunctive immunosuppressive agents, e.g. methotrexate, have been considered to allow reduction in use of glucocorticoids. Concomitant prescription of antiplatelet or anticoagulant therapy was suggested to reduce the occurrence of ischemic events in giant cell arteritis.
Dijagnostičke metode
The diagnosis of GCA can be made by a temporal artery biopsy or based on the association of demographic features (age > 50 years), clinical criteria (cranial symptoms), elevated inflammatory markers, and a favorable response to glucocorticoids. In temporal artery biopsy, the most important (and mandatory) histological criterion for the diagnosis of GCA is a mononuclear cell infiltrate predominating at the media-intima junction or involving the entire vessel wall (panarteritis). The potential role of imaging studies in the assessment of giant-cell arteritis (e.g., ultrasonography or MRI of the temporal arteries, F18 PET scan) has received increasing attention but it remains uncertain how they should best be integrated into the diagnostic workup.
Antenatalna dijagnoza
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Epidemiologija
GCA is the most common adulthood vasculitis with an annual incidence of 1/3,000-1/25,000 adults over 50 years old. It is more frequent in populations of northern European background. GCA affects people of more than 50 years old (median age at diagnosis between 70-75 years old) and occurs twice as frequently in women as in men.
Genetsko savetovanje
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Terapija
-
Klinička istraživanja
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