一种独特的 IgG4 疾病表现，伴有眼部、神经系统和乳突受累。
A unique presentation of IgG4 disease with ocular, neurologic and mastoid involvement.
机构信息
Division of Neurology, Stellenbosch University Faculty of Medicine and Health Sciences, Cape Town, Western Cape, South Africa
Neurology, Tygerberg Hospital, Cape Town, Western Cape, South Africa.
出版信息
BMJ Case Rep. 2022 Nov 14;15(11):e251736. doi: 10.1136/bcr-2022-251736.
A man in his 20s presented with headache and acute deterioration in visual acuity. He was found to have panuveitis and raised intracranial pressure with papilloedema. MRI and F-fluorodeoxyglucose positron emission tomography confirmed a subclinical, but active, inflammatory mastoid process. Histology of the mastoid showed immunoglobulin G4 (IgG4) cells, plasma cells and storiform fibrosis.This presentation of IgG4 disease has not been previously described.Treatment with high-dose steroids was initiated, followed by long-term immunosuppressive therapy. The patient's symptoms improved, although he remains dependent on azathioprine and low dose oral steroids for symptom control. To date, there has been no progression of the disease.
一位 20 多岁的男性出现头痛和视力急剧下降。他被发现患有全葡萄膜炎和伴有视乳头水肿的颅内压升高。MRI 和 F-氟代脱氧葡萄糖正电子发射断层扫描证实了亚临床但活跃的炎症性乳突过程。乳突组织学显示 IgG4 细胞、浆细胞和席纹状纤维化。这种 IgG4 疾病的表现以前没有描述过。开始使用大剂量类固醇治疗，随后进行长期免疫抑制治疗。患者的症状有所改善，但仍依赖硫唑嘌呤和低剂量口服类固醇来控制症状。迄今为止，疾病没有进展。
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