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Telitacicept for the treatment of Sjögren’s disease complicated by unilateral vocal fold immobility, severe hypergammaglobulinemia, and diabetes: a case report

Year: 2026

Type: article

Abstract: 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.

Source: Frontiers in Immunology

DOI: 10.3389/fimmu.2026.1956268