Lučić Aleksandra Tomić, Veselinović Mirjana, Pantović Suzana, Petrović Dejan, Zivanović Sandra, Milovanović Jasmina
Srp Arh Celok Lek. 2015 Jan-Feb;143(1-2):83-6.
Granulomatosis with polyangitis (Wegener's) is an antineutrophil cytoplasmic antibody (PR3-ANCA)-associated vasculitis, which commonly involves the upper and lower respiratory tracts and kidneys. Central nervous system involvement is reported in less than 11%, and rarely present at onset.
We report the case of a 41-year-old male patient with a high disease activity, large organ involvement, as well as central nervous system manifestations presented at onset.Treatment with intravenous pulse methylprednisolone, followed by the pulsed doses of cyclophosphamide was induced. After 6 months of cyclophosphamide pulse therapy a remission was achieved. Next, azathioprine was used for maintenance during the next 18 months.There were no disease flares during 24-month follow-up.
Granulomatosis with polyangitis (Wegener's) with large organ involvement, affecting the central nervous system structures require a rapid diagnosis and intensive medication treatment in order to prevent or reduce irreversible damage. Our experience confirms the findings reported in the literature that the severe forms of the disease are associated with increased probability of achieving remission, which reflects increased responsiveness of such patients to immunosuppressant therapy.
肉芽肿性多血管炎（韦格纳氏）是一种抗中性粒细胞胞浆抗体（PR3-ANCA）相关的血管炎，通常累及上、下呼吸道和肾脏。据报道，中枢神经系统受累的情况不到11%，且很少在发病时出现。
我们报告一例41岁男性患者，疾病活动度高，累及多个大器官，且发病时即出现中枢神经系统表现。诱导治疗采用静脉注射甲泼尼龙冲击治疗，随后给予环磷酰胺脉冲剂量治疗。环磷酰胺脉冲治疗6个月后病情缓解。接下来，在随后的18个月中使用硫唑嘌呤进行维持治疗。在24个月的随访期间未出现病情复发。
累及大器官、影响中枢神经系统结构的肉芽肿性多血管炎（韦格纳氏）需要快速诊断和强化药物治疗，以预防或减少不可逆损伤。我们的经验证实了文献报道的结果，即疾病的严重形式与缓解可能性增加相关，这反映出此类患者对免疫抑制治疗的反应性增强。
