IgG4相关性疾病的临床病例评析

目的提高对IgG4相关性疾病颅内病变的认识。 方法描述1例IgG4相关性疾病（IgG4-RD）颅内病变患者的诊治过程，总结其临床、影像学、病理特点，诊断和鉴别诊断，治疗和预后。 结果22岁女性，头痛、左眼视力下降10个月。6个月前左眼失明，眼球固定，左眼球后占位，病理示大量淋巴浆细胞浸润，IgG4＋浆细胞>50/高倍视野，IgG4（＋）/IgG比值>10%，予足量激素及小剂量免疫抑制剂治疗病情维持原状。2个月前停药。半月前突发右眼视力下降伴头痛加重，5天前右眼失明伴烦渴多尿，高钠血症。头颅MRI提示右侧视神经、鼻窦、鞍区、左侧颞叶及邻近脑膜异常信号。脑脊液压力、细胞学、生化均正常。予大剂量激素冲击治疗，序贯减量，联合环磷酰胺冲击治疗，患者右眼视力逐步恢复至0.3，头痛完全缓解，尿量改善，血钠正常。40余天后复查头颅MRI本次新发病灶明显好转。 结论IgG4-RD颅内病变可同时累及多个部位，但不伴颅外表现，可出现病情快速进展，及时治疗可避免不可逆损害。

A case of IgG4-related intracranial disease

Objective To improve the understanding of IgG4-related intracranial disease.Methods The clinical,radiographic,and pathological features,diagnosis,differential diagnosis,treatment,and prognosis of IgG4-ralated intracranial diseases were summarized by describing the clinical course of one patient.Results A 22-year-old woman presented with headache and decreased vision of left eye 10 months ago.She lost vision of her left eye 6 months ago and her left eyeball was fixed.MRI suggested a spaceoccupying lesion in the left orbit.The histopathological features were dense lymphoplasmacytic infiltrate,IgG4+plasma cells>50/high-power field,and IgG4(+)/IgG>10%.Her symptoms did not change after treatment with full-dose glucocorticoid and low-dose immunosuppressant.She stopped her drugs 2 months ago.Her vision of right eye decreased suddenly half a month ago and her headache became more serious.She totally lost her vision of right eye and suffered from polydipsia,polyuria,and hypernatremia 5 days ago.MRI suggested abnormal signals in the right optic nerve,paranasal sinuses,sellar region,left temporal lobe,and adjacent meninges.The examination of cerebral spinal fluid(CSF)was normal.Her vision of right eye recovered to 0.3 gradually after high-dose glucocorticoid pulse therapy combined with cyclophosphamide pulse therapy.Her headache disappeared and urine volume decreased.Her serum sodium turned to normal.Forty days later,MRI was repeated,which revealed that the new-onset lesions were almost normal.Conclusion IgG4-related intracranial disease could involve many regions without extracranial symptoms.This disease may progress very quickly in some cases and timely treatment could avoid irreversible damage.