韦格内肉芽肿是一种原因未明的多系统性非特异性坏死性肉芽肿。其特征是主要侵犯上呼吸道及/或下呼吸道和肾脏。病变只局限于呼吸道,不伴肾受累者称局限型,同时伴肾受累者称全身型。前者临床上少见,预后比全身型好,但可突然转变为全身型。病理特点病理组织学的主要特点是小动脉及小静脉的坏死性血管炎及肉芽肿形成。全身任何器官均可被侵犯,但以上呼吸道、肺及皮肤最常见。文献报告除肾外,还可累及淋巴腺、肝、脾、消化道、心脏、前列腺、眼、副睾、膀胱、卵巢、腮腺、乳腺、关节、骨骼肌及脑等处。Kornblut 等报告47例中,呼吸道占100%、肾85%、眼55%、关节48%、皮肤肌肉40%、耳36%、心与心包17%、神经系统15%。呼吸 Wegener’s granulomatosis is a non-specific, nonspecific necrotizing granuloma. It is characterized by a major violation of the upper respiratory and / or lower respiratory tract and the kidneys. Lesions are confined to the respiratory tract, without involvement of the kidney called those localized, accompanied by involvement of the kidney who said the whole body. The former clinically rare, the prognosis is better than the whole body type, but can suddenly change to the whole body type. Pathological features The main features of histopathology is the necrosis of small arteries and small veins and granuloma formation. Any part of the body can be violated, but the most common respiratory, lung and skin. Reported in addition to the literature kidney, but also involving the lymph gland, liver, spleen, digestive tract, heart, prostate, eye, epididymis, bladder, ovary, parotid gland, breast, joint, skeletal muscle and brain. Kornblut and other reports of 47 cases, the respiratory tract accounted for 100%, 85% of the kidneys, 55% of the eyes, joints 48%, skin and muscle 40%, 36% of ears, heart and pericardium 17%, 15% of the nervous system. Breathe