Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitides

Authors:

Dušanka Martinović Kaliterna, Ivanka Marinović, Ilza Salamunić

Summary

The anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides are a group of uncommon diseases characterised by inflammatory cell        infiltration and necrosis of blood vessel walls. ANCA there has been considerable progress towards understanding their pathogenesis. This results in endothelial activation with increased transmigration and adherence of neutrophils to vessel walls. Specific for granulomatosis with polyangiitis
are nasal or oral inflammation and development of oral ulcers and purulent or bloody nasal discharge. The chest radiograph usually showed the presence of nodules or fixed infiltrates. Microscopic polyangiitis affects the smallest blood vessels and may also affect mediumsized vessels, demonstrates the tropism for the kidneys – glomerulonephritis and lungs – pulmonary capillaritis. The characteristic features of eosinophilic granulomatosis with polyangiitis are asthma, eosinophilia in peripheral blood, sinusitis and pulmonary infiltrates which may be transient, than mononeuritis multiplex. It is important to differentiate ANCA vasculitis and syndromes that may mimic them, particularly infection, malignancy
and connective tissue disease.

Sažetak
Vaskulitisi malih krvnih žila, koji su povezani s antineutrofilnim citoplazmatskim protutijelima (ANCA), skupina su rijetkih bolesti koje obilježava    infiltracija upalnim stanicama i posljedična nekroza stijenki krvnih žila. Smatra se da ANCA imaju značajnu ulogu u patogenezi ovih bolesti. Dokazano je da potiču endotelnu aktivaciju te pojačavaju migraciju i adherenciju neutrofila za stijenku krvne žile. Obilježje granulomatoznog poliangiitisa je nastajanje nekrotizirajućih granuloma gornjeg i donjeg dijela respiratornog sustava. Najčešća radiološka obilježja na plućima sufiksni infiltrati, noduli i kavitacije. Mikroskopski poliangiitis zahvaća bubrege - glomerulonefritis i pluća - kapilaritis, a može zahvatiti i srednje velike krvne žile. Značajke eozinofi lne granulomatoze s poliangiitisom su astma, periferna eozinofilija, eozinofilna infiltracija tkiva, najčešće pluća, te mononeuritis multiplex. Diferencijalno dijagnostički važno je razlikovati ANCA vaskulitise od infekcija, malignih bolesti i bolesti vezivnog tkiva.

Vol.: 60

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