Yoshida M
Renal Unit of Internal Medicine, Hachioji Medical Center of Tokyo Medical College.
Nihon Rinsho. 1994 Aug;52(8):2089-94.
Wegener's granulomatosis (WG) is a multiorgan disease with a predilection for the upper airways (E: ear, eye, nose, throat) and lower airways (L: lung) and the kidneys (K):ELK classification. Typical histological features of WG include inflammation of small and medium sized vessels, necrosis and granuloma formation. The histological findings of WG are divided into 4 stages ie, I: degeneration II: acute inflammation III: granulation IV: scar. The recognition of the association between C (proteinase-3) antineutrophil cytoplasmic antibody (ANCA) and WG is the only tool available to aid in the assessment of disease activity, other than crude laboratory parameters of inflammation, such as, erythrocyte sedimentation rate and C reactive protein. Stage adapted immunosuppressive therapy (glucocorticoids and cyclophosphamide) has significantly altered the outcome of this once fatal disease.
韦格纳肉芽肿（WG）是一种多器官疾病，好发于上呼吸道（E：耳、眼、鼻、喉）、下呼吸道（L：肺）和肾脏（K），即ELK分类。WG的典型组织学特征包括中小血管炎症、坏死和肉芽肿形成。WG的组织学表现分为4期，即I：变性期；II：急性炎症期；III：肉芽组织期；IV：瘢痕期。除了炎症的粗略实验室参数，如红细胞沉降率和C反应蛋白外，识别抗中性粒细胞胞浆抗体（ANCA）与C（蛋白酶-3）之间的关联是目前可用于评估疾病活动度的唯一工具。根据分期调整的免疫抑制治疗（糖皮质激素和环磷酰胺）显著改变了这种曾经致命疾病的预后。
