Good综合征合并免疫介导性内耳病及系统性硬皮病1例并文献复习

Good Syndrome Complicated with Immune-Mediated Inner Ear Disease and Systemic Sclerosis: A Case Report and Literature Review

摘要: 回顾性分析该院血液内科收治的1例Good综合征(GS)合并免疫介导性内耳病(IMIED)及系统性硬皮病(SSc)患者的临床资料及治疗措施。患者，男，32岁，因右侧听力下降就诊，既往有胸腺瘤手术史，经检查确诊为Good综合征，结合听力检查及病因排除，考虑合并IMIED，病程中患者出现系统性硬皮病。患者接受药物保守治疗、糖皮质激素冲击及免疫球蛋白补充等综合治疗后，患者感染及免疫缺陷病情较前好转，听力损害未完全恢复，后患者因感染于院外死亡。对于存在免疫缺陷基础的患者，若出现突发性听力下降或皮肤纤维化等症状，需考虑合并IMIED或SSc的可能，为临床提供了一定的借鉴意义。

Abstract: This study retrospectively analyzed the clinical data and treatment of a patient with Good syndrome (GS) complicated with immune-mediated inner ear disease (IMIED) and systemic sclerosis (SSc) admitted to the Department of Hematology of our hospital. A 32-year-old male presented with right-sided hearing loss and had a history of thymoma surgery. The patient was diagnosed with GS after comprehensive examinations. Combined with audiological tests and exclusion of other etiologies, IMIED was considered as a complication. During the disease course, systemic sclerosis developed. The patient received comprehensive treatments including conservative drug therapy, glucocorticoid pulse therapy, and immunoglobulin supplementation, which resulted in improved infection and immunodeficiency conditions; however, hearing impairment was not fully recovered. The patient eventually died of infection outside the hospital. For patients with underlying immunodeficiency, the possibility of concurrent IMIED or SSc should be considered when sudden hearing loss or skin fibrosis occurs. This case provides valuable clinical insights.