A case of granulomatosis with polyangiitis (Wegener's granulomatosis) manifested with asymptomatic intracerebral hemorrhage-猫眼课题宝

DOI：

10.2177/jsci.36.58

发表时间：

2013-02

期刊：

Japanese Journal of Clinical Immunology

影响因子：

通讯作者：

中科院分区：

其他

文献类型：

作者：

H. Takaoka;A. Hashimoto;S. Nogi;K. Iwata;H. Futami;Y. Arinuma;K. Shimada;H. Nakayama;A. Komiya;H. Furukawa;T. Matsui;S. Tohma

患者男，46岁，鼻窦炎，出现双侧耳痛、下肢瘀点和右眼充血。血液检查发现血清C反应蛋白水平升高。计算机断层扫描偶然发现丘脑出血的急性病变，无神经症状，当时未给予特殊治疗。此后，他出现眩晕、呕吐和肺炎，抗生素对其无效。他被转诊到我们医院。此外，耳部和肾脏病变以及血清蛋白酶3-抗神经细胞胞浆抗体（PR 3-ANCA）的存在证实了他对肉芽肿伴多血管炎（韦格纳氏）（GPA）的诊断。在皮质类固醇和环磷酰胺治疗下，他的症状在两个月内消失沿着PR 3-ANCA消退。之后，他没有表现出新的大脑病变或症状。这是一例罕见的以无症状性脑出血为表现的GPA病例。值得注意的是，GPA可引起中枢神经系统的各种表现，如致命或无症状的出血性病变，这可能对免疫抑制剂治疗有反应。

: A 46-year-old man, who had had sinusitis, developed bilateral omalgia, petechiae on his lower extremities and a congested right eye. A blood test detected elevated serum C-reactive protein level. Computed tomography incidentally found an acute lesion of thalamic hemorrhage without neurological symptoms and no specific therapy was given at the time. Thereafter, he developed vertigo, vomiting and pneumonia for which antibiotics were ineffective. He was referred and admitted to our hospital. Further, aural and renal lesions, and presence of serum proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) confirmed his diagnosis of granulomatosis with polyangiitis (Wegener's) (GPA). With corticosteroid and cyclophosphamide therapy, his symptoms disappeared in two months along with faded PR3-ANCA. Afterward he showed neither new cerebral lesion nor symptom. This is a rare case of GPA manifested with asymptomatic intracerebral hemorrhage. It should be noted that GPA could cause various manifestations in central nervous system such as a fatal or an asymptomatic hemorrhagic lesion, which might respond to immunosuppressive therapy.

A case of granulomatosis with polyangiitis (Wegener's granulomatosis) manifested with asymptomatic intracerebral hemorrhage

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