样坏死性小血管炎。肾活检见肾小球毛细血管袢纤维素样坏死和新月体形成。肺活检可见肺毛细血管炎或肉芽肿病变。鉴别诊断 :应与结节性多动脉炎、狼疮肾炎及肺出血 肾炎综合征 (Goodpasture ssyndrome)等相鉴别。本病目前出现误诊和漏诊较为突出 ,主要原因是对本病认识不足 ,医生没有将患者全身多脏器损害的表现 ,全面综合分析 ,从一元论的角度寻找病因。常常将该病的不同阶段出现的症状孤立起来处理 ,如患者出现鼻窦炎、咽溃疡、眼炎 ,则在五官科就诊处理 ;出现肺症状 ,就到呼吸内科处理 ,到出现蛋白尿、血尿、肾衰竭时才到肾科诊治 ,甚至到了肾科也不一定能够及时确诊。有些病人因关节痛、肌痛突出就诊于风湿内科也长期误诊为反应性关节炎 ,最后发展为咯血、肾衰竭 ,才检测出抗中性粒细胞胞浆抗体阳性而确诊 ,此时因疾病已累及重要器官难以逆转 ,但经治疗后仍可缓解 ,因此对本类病应在未出现重要器官受累之前明确诊断 ,及时应用免疫抑制药治疗 ,可明显改善患者的预后。4　治　疗诱导期首选肾上腺皮质激素 (激素 )加环磷酰胺联合治疗。泼尼松开始应用每日 1mg/kg,共服 4～ 8周 ,以后逐渐减量至 5～ 10mg/d ,维持 2年或更长。重症肾衰竭或肺病变严重者可用甲泼尼龙冲击治疗。环磷酰胺可口服 ,每日 2mg/ Necrotic small vasculitis. Renal biopsy see glomerular capillary tumefial necrosis and crescent formation. Pulmonary biopsy showed pulmonary capillary granuloma or granuloma lesions. Differential diagnosis: should be with polyarteritis nodosa, lupus nephritis and pulmonary hemorrhagic nephritic syndrome (Goodpasture ssyndrome) phase identification. The current misdiagnosis and missed diagnosis is more prominent, mainly due to lack of understanding of the disease, the doctor did not show the performance of patients with multiple organ damage comprehensive and comprehensive analysis of the causes from a monistic look. Often the symptoms of different stages of the disease appear isolated treatment, such as patients with sinusitis, pharyngeal ulcer, ophthalmia, treatment at the ENT treatment; pulmonary symptoms, to respiratory medicine treatment, to appear proteinuria, hematuria, Renal failure to renal surgery, and even to the nephrology may not be able to timely diagnosis. Some patients due to joint pain, myalgia prominent rheumatology attending rheumatoid arthritis also misdiagnosed as long-term, and finally developed into hemoptysis, renal failure, was detected positive anti-neutrophil cytoplasmic antibodies and diagnosed at this time because the disease has been Involved in the vital organs difficult to reverse, but after treatment can still be alleviated, so the type of disease should be clear in the absence of an important organ involvement before diagnosis, timely application of immunosuppressive drugs can significantly improve the prognosis of patients. 4 treatment induction period preferred adrenal cortex hormone (hormone) plus cyclophosphamide combination therapy. Prednisone started daily application of 1mg / kg, a total of 4 to 8 weeks, then gradually reduced to 5 ~ 10mg / d, for 2 years or longer. Serious cases of severe renal failure or pulmonary disease may be treated with methylprednisolone. Cyclophosphamide can be taken orally, daily 2mg /