MINI REVIEW article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
A running title: primary Sjögren's Syndrome-Associated Immune Thrombocytopenia: From Pathogenesis to Treatment
Chang Liu 1
Ziqiang Zheng 1,2
Taoyuan He 2
GuiXiang Zhang 3
GuoHua Fu 3
1. Department of Rheumatology and Immunology, Central Hospital of Dalian University of Technology, Dalian, China
2. Dalian Medical University, Dalian, China
3. The Second Hospital of Longyan, Longyan, China
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Abstract
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.
Summary
Keywords
immune thrombocytopenia, platelets, Predictive factors, primary Sjögren's syndrome, Treatment
Received
17 November 2025
Accepted
17 February 2026
Copyright
© 2026 Liu, Zheng, He, Zhang and Fu. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
*Correspondence: Chang Liu
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