Primary Sjögren’s syndrome-associated immune thrombocytopenia: from pathogenesis to treatment

Primary Sjögren’s syndrome (pSS) is an autoimmune disorder characterized by xerostomia and keratoconjunctivitis sicca, with approximately 10%–20% of patients developing concurrent immune thrombocytopenia (ITP). Recent studies suggest the pathogenesis of primary Sjögren’s syndrome - associated immune thrombocytopenia (pSS-ITP) may involve dysregulated TLR7 signaling, B-cell hyperactivation, and autoantibody-mediated platelet destruction. Beyond conventional therapies (e.g., glucocorticoids and intravenous immunoglobulin [IVIG]), emerging treatments have garnered increasing attention, including thrombopoietin receptor agonists (TPO-RAs), B-cell–targeted therapies, and mTOR inhibitors. Predictive models incorporating bone marrow megakaryocyte counts and autoantibody profiles may facilitate individualized treatment selection. Future multicenter clinical studies are warranted to evaluate the long-term efficacy and safety of novel agents and to explore biomarker-guided precision therapy. This review systematically summarizes the pathophysiological mechanisms of pSS- ITP, synthesizes current clinical treatment strategies, and highlights key biomarkers with potential implications for therapeutic response, aiming to provide a theoretical foundation and practical guidance for optimizing individualized therapeutic regimens.