Perioperative B-Cell-Targeted Therapy for High-Risk Valve Surgery in Active Primary Sjögren's Disease

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.

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.

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.