Key result
Telitacicept-based perioperative regimen enables successful aortic valve replacement with durable 1-year function.
Why the study?
Clinically significant valvular involvement in primary Sjögren's disease is uncommon and evidence guiding perioperative immunomodulation is limited.
Case Report (n=1)
A multidisciplinary perioperative strategy incorporating B-cell-targeted therapy facilitated a successful valve replacement and recovery in a patient with severe valvular heart disease and active primary Sjögren's disease.
Multidisciplinary perioperative telitacicept use appears feasible in active SjD with valve replacement; leaves open standardized immunomodulation protocols.
BACKGROUND: Primary Sjögren's disease (SjD) is a systemic autoimmune disease driven by B-cell dysregulation. Clinically significant valvular involvement is uncommon, and evidence guiding perioperative immunomodulation remains limited. CASE SUMMARY: A 59-year-old woman with SjD and severe aortic stenosis required valve replacement. Preoperative evaluation demonstrated active SjD with elevated inflammatory markers and hypergammaglobulinemia. Following multidisciplinary discussion, telitacicept, corticosteroids, and hydroxychloroquine were initiated before surgery. After improvement in inflammatory activity, the patient underwent aortic valve and ascending aorta replacement. Histopathologic examination showed degenerative valvular changes with focal inflammatory cell infiltration. The postoperative course was uneventful, with normal prosthetic valve function at 1-year follow-up. DISCUSSION: This case highlights the challenges of managing severe valvular heart disease in patients with active primary SjD and a multidisciplinary perioperative strategy incorporating B-cell-targeted therapy. Although preoperative immunomodulation may have attenuated the histopathologic evidence of immune-mediated valvular involvement, the case offers insights when guidance is limited.
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Li et al. (2026) conducted a case report in Primary Sjögren's disease and severe aortic stenosis (n=1). Perioperative B-cell-targeted therapy (telitacicept, corticosteroids, and hydroxychloroquine) and aortic valve replacement was evaluated. Perioperative B-cell-targeted therapy using telitacicept, corticosteroids, and hydroxychloroquine enabled successful aortic valve replacement in a 59-year-old woman with normal function at 1 year.
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