MEDIX, God. 20 Br. 113/114  •  Pregledni članak  •  Reumatologija HR ENG

ANCA-vaskulitisi – novosti u dijagnostici i liječenjuANCA-associated vasculitides – advances in the diagnosis and treatme

Dušanka Martinović Kaliterna, Daniela Marasović Krstulović

Vaskulitisi povezani s antineutrofilnim citoplazmatskim protutijelima (ANCA) skupina su rijetkih bolesti obilježenih upalnom infiltracijom stijenki krvnih žila, posljedičnom nekrozom stijenki te disfunkcijom zahvaćenih organa. Poznato je da ANCA imaju ulogu u patogenezi potičući endotelnu aktivaciju te migraciju i adherenciju neutrofila za stijenku krvne žile. ANCA-povezani vaskulitisi (engl. (ANCA-associated vasculitides – AAV) obuhvaćaju tri entiteta: granulomatozni poliangitis (GPA), mikroskopski poliangitis (MPA) te eozinofilnu granulomatozu s poliangitisom (EGPA), a razlikuju se prema kliničkim obilježjima, tropizmu za tkiva i organe te različitoj serološkoj pojavnosti antineutrofilnih citoplazmatskih protutijela (ANCA). Najčešće zahvaćeni organi su koža, dišni putevi, pluća, bubrezi te periferni živci. Suvremeno liječenje teških oblika AAV-a uključuje, uz konvencionalnu terapiju glukokortikoidima i ciklofosfamidom, rituksimab, osobito za relapsni oblik bolesti. 

Ključne riječi:
antineutrofilna citoplazmatska protutijela; granulomatoza; poliangitis; upala; vaskulitisi

Članak u cijelosti pročitajte u tiskanom izdanju MEDIX, God. 20 Br. 113/114

Vasculitides associated with antineutrophil cytoplasmic antibodies (ANCA) are a group of rare disorders characterized by inflammatory infiltration of the vascular wall, subsequent necrosis of the walls and dysfunction of the affected organs. It is known that ANCA has a role in the pathogenesis of stimulating endothelial activation and migration and adherence of neutrophils to the vascular wall. ANCA associated vasculitis (AAV) comprises three entities: granulomatous polyangiitis (GPA), microscopic polyangitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), which differ according to clinical features, tissue and organ tropism, and different serological prevalences of ANCA. The most commonly affected organs are the skin, respiratory tract, lungs, kidneys and peripheral nerves. In addition to conventional therapy with glucocorticoids and cyclophosphamide, treatment of severe forms of AAV includes rituximab, especially for the relapsing form of the disease. 

Key words:
antineutrophil cytoplasmic antibodies; granulomatosis; inflammation; poliangitis; vasculitis