泰它西普治疗干燥病合并单侧声带固定、重度高丙种球蛋白血症及糖尿病的病例报告

文献信息

中文摘要

背景： 干燥病（SjD）涉及多种腺体外表现（EGMs）。重度高丙种球蛋白血症及疑似与喉返神经（RLN）相关的单侧声带固定代表侵袭性表型。此类表现的处理可能受糖尿病等代谢合并症限制，因为后者限制了常规大剂量糖皮质激素的使用。方法：本文描述一名48岁女性SjD合并2型糖尿病（T2DM）患者，表现为重度高丙种球蛋白血症（IgG 38.9 g/L）、急性声嘶及干燥症状。喉频闪镜检查显示左侧单侧声带固定，可能提示神经源性麻痹。正电子发射断层扫描-计算机断层扫描（PET-CT）未发现沿可见RLN走行的明显肿块或外压，降低了压迫性或恶性病因的可能性。EULAR干燥综合征疾病活动指数（ESSDAI）评分为26。结果：启动包含泰它西普（160 mg/周）、甲泼尼龙（20 mg/日）和羟氯喹（0.2 g，每日两次）的联合治疗方案，既为尽量减少糖皮质激素暴露，也为针对患者以重度高丙种球蛋白血症为表现的显著B细胞高活性。治疗期间观察到明显的血清学和症状改善。15周内，IgG水平降至14.6 g/L，类风湿因子（RF）从759 IU/mL降至58.3 IU/mL，ESSDAI评分降至6。患者声嘶症状改善，同时甲泼尼龙成功减量至4 mg/日。基于患者报告症状的临床稳定性持续至12个月随访。结论：本病例具有假设生成意义：含泰它西普的方案可能对伴喉部/声带受累的高活动性SjD产生明显血清学改善和症状获益，同时降低糖皮质激素相关代谢风险。确切疗效需在更大规模对照研究中确认。

英文摘要

Sjögren's Disease (SjD) involves diverse extraglandular manifestations (EGMs). Severe hypergammaglobulinemia and suspected recurrent laryngeal nerve (RLN) -related unilateral vocal fold immobility represent aggressive phenotypes. Management of such presentations may be constrained by metabolic comorbidities such as diabetes, which limit the use of conventional high-dose glucocorticoids. Herein, we describe a 48-year-old female with SjD and Type 2 Diabetes Mellitus (T2DM) who presented with severe hypergammaglobulinemia (IgG 38.9 g/L), acute hoarseness, and sicca symptoms. Laryngostroboscopy demonstrated left-sided unilateral vocal fold immobility, possibly suggestive of neurogenic paralysis. Positron emission tomography-computed tomography (PET-CT) did not identify obvious masses or extrinsic compression along the visualized RLN course, reducing the likelihood of a compressive or malignant etiology. The EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) score was 26. A combined therapeutic regimen comprising telitacicept (160 mg/week), methylprednisolone (20 mg/day) and hydroxychloroquine (0.2 g, twice daily) was initiated, both to minimize glucocorticoid exposure and to target the patient's profound B-cell hyperactivity manifested by severe hypergammaglobulinemia. Marked serological and symptomatic improvements were observed during treatment. Within 15 weeks, the IgG level decreased to 14.6 g/L, rheumatoid factor (RF) decreased from 759 IU/mL to 58.3 IU/mL, and the ESSDAI score dropped to 6. The patient experienced symptomatic improvement of hoarseness, while methylprednisolone was successfully tapered to 4 mg/day. Clinical stability based on patient-reported symptoms persisted through the 12-month follow-up. This case is hypothesis-generating: A telitacicept-containing regimen may produce marked serological improvement and symptomatic benefit for high-activity SjD with laryngeal/vocal fold involvement, while reducing glucocorticoid-related metabolic risks. Definitive efficacy requires confirmation in larger controlled studies.