韦格纳肉芽肿的CT表现和诊断进展

CT manifestations and diagnostic progress of Wegener's granulomatosis

韦格纳肉芽肿是一种病因不太明确的自身免疫性疾病,以低发病率和高死亡率为特点。主要病理特征为肉芽肿、局灶性坏死和血管炎三联征,主要累及小动脉、毛细血管和小静脉。病变可累及多种组织和器官,通常以上下呼吸道、肾脏、皮肤及眼眶最为常见。研究表明其发病受环境因素、感染及细胞免疫因素的影响。该病起病隐匿,临床症状无特异性,大部分患者以上呼吸道症状为首发表现,部分可见咳血、呼吸困难等症状,典型者有上呼吸道、肺、肾三联征。CT表现具有多发性、多样性、多变性的"三多"典型影像学特点,其循环内抗中性粒细胞胞质抗体(ANCA)也存在一定的特异性,目前较为有效的临床药物为糖皮质激素和免疫抑制剂联合应用,临床上难治性病例也考虑TNF-α抑制剂等生物制剂的应用。鉴于其临床表现及诊断学方面的非特异性,临床上漏诊及误诊率较高,因此早期诊断和治疗对预后具有重要意义。

Wegener＇s granulomatosis is a kind of autoimmune disease whose etiology is not clear with the characteristics of low morbidity and high mortality. Main pathological features are granuloma, focal necrosis and vasculitis views, mainly involving the small arteries, capillaries and venules. Lesions can involve a vari- ety of tissues and organs, and upper and lower respiratory tract, kidney, skin and eyes are most common. Studies have shown that the disease is affected by environmental factors, infection, and cellular immune fac- tors. The disease onset conceals, lack of characteristic clinical symptoms. Most patients has respiratory symp- toms for starting performance, the visible part of hemoptysis, dyspnea and other symptoms, typical ones has the triad of upper respiratory tract, lung, kidney views. CT manifestations hare typical imaging features of multiple, diversity and changeability, its circulation within the anti neutrophil cytoplasmic antibodies （ANCA） also has certain specificity, the more effective clinical drugs are the joint application of glucocorticoid and immunosuppressants, clinical refractory cases also consider the application of the biological agents such as TNF alpha inhibitors. In view of its nonspecific clinical manifestations and diagnosis, clinical diagnosis and the misdiagnosis rate is high, so early diagnosis and treatment is of great significance to the prognosis.