Kaliterna Dusanka Martinović, Marinović Ivanka, Salamunić Iliza
Reumatizam. 2013;60(2):43-6.
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 medium-sized 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.
抗中性粒细胞胞浆抗体(ANCA)相关血管炎是一组罕见疾病,其特征为炎症细胞浸润和血管壁坏死。在理解其发病机制方面已经取得了相当大的进展。这导致内皮细胞活化,中性粒细胞向血管壁的迁移和黏附增加。肉芽肿性多血管炎的特异性表现为鼻或口腔炎症、口腔溃疡的发生以及脓性或血性鼻分泌物。胸部X线片通常显示有结节或固定浸润影。显微镜下多血管炎累及最小的血管,也可能累及中等大小血管,表现为对肾脏——肾小球肾炎和肺部——肺毛细血管炎的嗜性。嗜酸性肉芽肿性多血管炎的特征性表现为哮喘、外周血嗜酸性粒细胞增多、鼻窦炎和肺部浸润(可能是短暂性的),而非多发性单神经炎。区分ANCA血管炎和可能与之相似的综合征非常重要,尤其是感染、恶性肿瘤和结缔组织病。