Arteritis divovskih stanica

Dijana Perković, Simeon Grazio, Tatjana Kehler, Jadranka Morović Verglas, Srđan Novak, Višnja Prus, Branimir Anić

Arteritis divovskih stanica ili gigantocelularni arteritis (engl. Giant Cell Arteritis – GCA) najčešći je vaskulitis starije životne dobi. Obično zahvaća ogranke karotidnih arterija, poglavito temporalnu i oftalmičku arteriju. Nastanku bolesti uz genetsku predispoziciju doprinose i okolišni čimbenici. Interleukin 6 (IL-6), čija razina korelira s upalnom aktivnošću, jedan je od ključnih citokina u patogenezi bolesti. Sterilna upala stijenke arterije uz hiperplaziju intime i razvoj okluzije dovodi do ishemije koja je odgovorna za nastanak simptoma. U kliničkoj slici dominira glavobolja uz opće simptome. Može nastati i nagli gubitak vida zbog anteriorne ishemičke optičke neuropatije (engl. anterior ischemic optic neuropathy – AION). Nerijetko su prisutni i znakovi reumatske polimijalgije (polymyalgia rheumatica – PMR). U laboratorijskim nalazima tipična je izrazito ubrzana sedimentacija eritrocita (SE) i povišen C reaktivni protein (CRP). Dijagnoza se temelji na kliničkoj slici, laboratorijskim nalazima, patohistološkoj analizi bioptata temporalne arterije te radiološkim slikovnim prikazima. Brzo postavljanje dijagnoze ključno je za pravodobno i adekvatno liječenje i sprječavanje nastanka ranih i kasnih komplikacija (sljepoće, aneurizme i disekcije arterija). Liječenje je donedavno bilo ograničeno na primjenu glukokortikoida (GK) i konvencionalnih imunomodulatora. U ovom preglednom radu navedene su nove terapijske mogućnosti proizašle iz boljeg poznavanja patofiziologije bolesti. Recentna istraživanja rezultirala su korekcijama smjernica za liječenje GCA (što je 2018. godine učinilo i Hrvatsko reumatološko društvo) preporukom primjene IL-6 inhibitora, tocilizumaba u bolesnika s refraktornom ili relapsnom bolešću, a inicijalno u bolesnika s povišenim rizikom za razvoj komplikacija. U tijeku su klinička istraživanja koja otvaraju i druge terapijske opcije u skoroj budućnosti.

Giant cell arteritis (GCA) is the most common vasculitis of older age. It usually affects the branches of carotid arteries, especially temporal and ophthalmic artery. In addition to genetic predisposition, environmental factors also contribute to the development of the disease. Interleukin 6 (IL-6), whose level correlates with inflammatory activity, is one of the key cytokines in the pathogenesis of the disease. Sterile inflammation of the artery wall with intimal hyperplasia and the development of occlusion leads to ischemia which is responsible for the onset of symptoms. The clinical picture is dominated by headache together with general symptoms. Sudden loss of vision may occur due to anterior ischemic optic neuropathy (AION). Frequently, signs of rheumatic polymyalgia are also present. Markedly high erythrocyte sedimentation rate (ESR) and elevated C reactive protein (CRP) are typical. The diagnosis is based on clinical and laboratory findings, temporal artery biopsy, and imaging examinations. Prompt diagnosis is crucial for timely and adequate treatment as well as prevention of early and late complications (blindness, arterial aneurysm or dissection). Until recently, treatment has been limited to the use of glucocorticoids and conventional immunomodulating drugs. This review paper outlines new therapeutic approaches arising from a better knowledge of pathophysiology. Insights from recent studies led to corrections of
GCA treatment guidelines (as did the Croatian Society for Rheumatology in 2018) by recommending IL-6 inhibitor tocilizumab for patients with refractory or relapsing disease, and initially in patients at increased risk for complications. Clinical trials are underway that could deliver other therapeutic options in the near future.