韦格纳肉芽肿合并眶内假瘤13例临床分析

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...

Clinical analysis of 13 patients of wegener granulomatosis with orbital pseudotumor

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.