de Groot K, Gross W L
Medizinische Universität Lübeck, Abteilung Klinische Rheumatologie, Bad Bramstedt, Germany.
Lupus. 1998;7(4):285-91. doi: 10.1191/096120398678920118.
Wegener's granulomatosis (WG) belongs to the group of necrotizing primary systemic vasculitides of unknown etiology, that are associated with anti-neutrophil cytoplasmic antibodies. The pathological hallmark of WG is the coexistence of vasculitis and granuloma. Due to more sensitive diagnostic instruments, especially ANCA testing, the incidence of diagnosis of WG has risen in the past ten years. Although the precise pathophysiology is not understood yet, there is ample evidence that ANCA, which can lead to cytotoxic reactions in the vascular texture, play a major role, possibly promoted by a dysbalance in the anti-idiotypic network. The clinical disease course is typically two-phasic, beginning with a granulomatous inflammation of the upper respiratory tract, that usually is followed by a generalized vasculitic phase, that can range from mild organ dysfuntion to life threatening multi-organ failure. Consequently, diagnostic procedures, patients' assessment and therapeutic regimens need to be individualized, adapted to stage and activity of the disease as well as standardized.
韦格纳肉芽肿（WG）属于病因不明的坏死性原发性系统性血管炎组，与抗中性粒细胞胞浆抗体相关。WG的病理标志是血管炎和肉芽肿并存。由于诊断工具更加灵敏，尤其是抗中性粒细胞胞浆抗体（ANCA）检测，在过去十年中，WG的诊断发病率有所上升。尽管尚未完全了解其确切的病理生理学，但有充分证据表明，可导致血管组织发生细胞毒性反应的ANCA发挥着主要作用，抗独特型网络失衡可能会促进这种作用。临床病程通常分为两个阶段，始于上呼吸道的肉芽肿性炎症，随后通常会进入全身性血管炎阶段，其范围可从轻度器官功能障碍到危及生命的多器官衰竭。因此，诊断程序、患者评估和治疗方案需要个体化，根据疾病的阶段和活动情况进行调整并实现标准化。
