作者自1963至1983年共诊治进行性坏死性鼻病80余例,其中27例确诊为 Wegener 肉芽肿病(WG),30例确诊为恶性组织细胞增多症(MH)。就二者的诊断标准、临床表现、预后及治疗做一报告。WG 诊断标准:(1)上呼吸道有坏死性肉芽病变。(2)活检发现巨细胞肉芽或类纤维蛋白变性血管炎。(3)实验室检查:血沉快;α_2及γ球蛋白增多;C 反应蛋白阳性;类风湿因子阳性;血清补体效价 CH_(60)(50％)补体溶血单位)升高;嗜中性白细胞游走能力减弱;细胞免疫功能减退;血小板计数增加;小细胞性贫血;尿蛋白阳性。(4)特殊临床表现:鞍鼻,喉、声门下腔或气管上段环形狭窄;肾炎;胸片有阴影;水疱或紫癜等皮疹。(5)肾上腺皮质激素 The author diagnosed 80 cases of progressive necrotizing rhinopathy from 1963 to 1983, of which 27 cases were diagnosed as Wegener’s granulomatosis (WG) and 30 cases were diagnosed as malignant histiocytosis (MH). Make a report on the diagnostic criteria, clinical manifestations, prognosis and treatment of both. WG diagnostic criteria: (1) upper respiratory tract necrotic granulomatous lesions. (2) biopsy found that giant cell granulation or fibrinous vasculitis. (3) laboratory tests: ESR; α_2 and γ-globulin increased; C-reactive protein positive; rheumatoid factor positive; serum complement titer CH_ (60) (50%) complement hemolysis units); neutrophils Weakened walking ability; decreased cellular immunity; increased platelet count; small cell anemia; positive urine protein. (4) special clinical manifestations: saddle nose, larynx, subglottic or tracheal ring stenosis; nephritis; chest shadow; blisters or purpura and other rash. (5) Cortina