Response to telitacicept in optic neuritis associated with Sjogren's syndrome: a case report and literature review

Abstract

Background: Optic neuritis (ON) frequently presents as the initial symptom of multiple sclerosis and neuromyelitis optica. Additionally, ON is one of the manifestations of central nervous system damage in Sjögren's syndrome (SS). However, there is little research on SS-associated ON. Currently, there are no standard treatment methods for SS-associated ON, and the recurrence rate remains high.

Methods: A 54-year-old female patient primarily exhibited symptoms of dry mouth and dry eyes, along with rapidly deteriorating vision and pain in the left eye. Laboratory tests revealed strong positivity for anti-Sjögren's-syndrome-related antigen A and anti-Sjögren's-syndrome-related antigen B antibodies, and a labial salivary gland biopsy showed focal lymphocytic sialadenitis with a focus score of 13 per 4 mm². Visual evoked potentials indicated a significant absence of waveform in the left eye. The patient was diagnosed with SS-associated ON; however, treatment with glucocorticoid (GC) and cyclophosphamide was unsatisfactory. The patient declined plasma exchange and Rituximab therapy; consequently, she was treated with GC and telitacicept.

Results: During a 9-month follow-up period, at the minimum GC dosage, the patient's symptoms of dryness and visual acuity in the left eye improved significantly, with no reported recurrence or adverse drug reactions. This indicates both good clinical efficacy and safety.

Conclusion: SS-associated ON is uncommon and easily misdiagnosed. Conventional therapies for ON cannot control recurrence. Telitacicept might represent a promising choice to treat SS-associated ON.

Keywords: Optic neuritis; Sjögren’s syndrome; Telitacicept; Therapy.