韦格纳肉芽肿合并眶内假瘤13例临床分析  

Clinical analysis of 13 patients of wegener granulomatosis with orbital pseudotumor

作　　者：张国华[1] 吴庆军[1] 曾小峰[1] 张立民[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院风湿免疫科,100730

出　　处：《北京医学》2011年第3期216-220,共5页Beijing Medical Journal

摘　　要：目的总结分析13例韦格纳肉芽肿（wegener granulomatosis,WG）合并眶内假瘤患者的临床特点。方法回顾性分析北京协和医院2003年2月至2010年4月确诊的13例WG合并眶内假瘤患者的临床资料。结果 13例患者男女比例2.25∶1,年龄17～58岁,中位年龄28岁,眶内假瘤病程1~12个月,中位时间4个月。13例患者中11例伴突眼,眼的其他临床表现有巩膜炎及角膜炎各2例,失明1例;13例患者中12例为鼻窦或鼻咽部软组织占位。胞浆型抗中性粒细胞胞浆抗体（cANCA）阳性率为54%,ANCA阴性率为38%。3例眶内组织活检仅1例（33%）出现典型的病理三联征,6例鼻组织活检4例出现炎性肉芽肿具有诊断意义。糖皮质激素联合环磷酰胺治疗,诱导缓解期总缓解率为62%,2例难治型分别给予类克及美罗华治疗后,眼部症状明显改善。随访1~84个月,2例（20%）合并疱疹病毒感染,2例致眼球摘除,1例视力丧失,无死亡病例。结论 WG合并眶内假瘤不少见,多为鼻部肉芽肿侵犯眼眶所致,应提高警惕,同时提高对ANCA阴性WG的认识。难治型可予美罗华或类克等生物制剂治疗。Objective To investigate the clinical features of 13 cases with wegener granulomatosis（WG） complicated by orbital pseudotumor. Methods 13 patients of orbital pseudotumor with WG admitted to our center in recent 7 years were retrospectively analyzed. Results The ratio of male to female was 2.25∶1. The median age was 28 years （ranging from 17 to 58 years）. The median disease duration of rbital pseudotumor was 4 months（1~12 months）. Exophthalmos was present in 11 of 13 cases, and other ocular clinical manifestation included scleritis and keratitis in 2 cases respectively. 12 of 13 cases had nasal sinus or pharynx granuloma disease. The positive rate of cANCA was 54%, while the negative rate of ANCA was 38%. Only 1 of 3 patients with orbital lesion biopsy showed typical necrotic granuloma and microvasculitis. The pathologic inflammatory granuloma, which emerged in 4 of 6 patients with nasal tissue biopsy, could be a diagnostic clue. The remission rate in induction phase treated with corticosteroid and cyclophosphamide was 62%. Ocular symptoms improved in 2 refractory WG patients with rituximab and infliximab therapy respectively. The follow-up duration was from 1 to 84 months. Complications included herpesvirus infection（20%） in 2 cases, eyeball removal in 2 cases, and loss of sight in 1 patient. There was no death case. Conclusion We should pay attention to orbital pseudotumor invaded by nasal granuloma in WG. Moreover, ANCA negative WG should be taken into account. Biological agents, such as rituximab or infliximab, could be used to treat refractory WG.

关 键 词：韦格纳肉芽肿 眶内假瘤 治疗 

分 类 号：R593.9[医药卫生—内科学]