{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T06:20:20Z","timestamp":1783059620003,"version":"3.54.6"},"reference-count":46,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T00:00:00Z","timestamp":1783036800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>\n                    Sj\u00f6gren\u2019s syndrome (SS) is a systemic autoimmune disorder frequently associated with diverse hematological manifestations. Immune-mediated thrombotic thrombocytopenic purpura (iTTP) and macrophage activation syndrome (MAS) represent rare, life-threatening complications, yet the consecutive occurrence of both conditions in the same SS patient is exceedingly rare. We report a 20-year-old female diagnosed with SS following drug-induced liver injury. In July 2023, she developed acute iTTP (0% ADAMTS13 activity, with positive inhibitory antibodies) shortly after the initiation of immunomodulatory therapy, which responded to plasma exchange and corticosteroids. In December 2023, following\n                    <jats:italic>Mycoplasma pneumoniae<\/jats:italic>\n                    infection, she presented with MAS, fulfilling seven of eight hemophagocytic lymphohistiocytosis (HLH)-2004 criteria. Management included high-dose methylprednisolone, etoposide, and ruxolitinib, followed by plonmarlimab (anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) antibody) under a clinical trial protocol. This unique case underscores the complexity of SS-associated hematological emergencies. Based on our findings, we propose a dual-pathway hypothesis for these complications: firstly, elevated IL-6 may facilitate the thrombotic cascade in SS-associated iTTP; secondly, the interferons-Janus kinases-signal transducer and activator of transcription (IFN-JAK-STAT) pathway\u2019s aberrant activation likely creates a mechanistic intersection that predisposes SS patients to MAS. While the efficacy of targeted agents like tocilizumab, ruxolitinib, and plonmarlimab appears promising in this context, these therapeutic avenues strictly warrant further systematic investigation through larger clinical cohorts.\n                  <\/jats:p>","DOI":"10.3389\/fimmu.2026.1810384","type":"journal-article","created":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T05:39:27Z","timestamp":1783057167000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Thrombotic thrombocytopenic purpura and macrophage activation syndrome secondary to Sj\u00f6gren\u2019s syndrome: a case report"],"prefix":"10.3389","volume":"17","author":[{"given":"Xiaoya","family":"Liu","sequence":"first","affiliation":[{"name":"The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital, School of Clinical Medicine, Shandong First Medical University","place":["Jinan, Shandong, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maiqi","family":"Liu","sequence":"additional","affiliation":[{"name":"The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital, School of Clinical Medicine, Shandong First Medical University","place":["Jinan, Shandong, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mengjiao","family":"Yao","sequence":"additional","affiliation":[{"name":"The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital, School of Clinical Medicine, Shandong First Medical University","place":["Jinan, Shandong, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haohu","family":"Wu","sequence":"additional","affiliation":[{"name":"The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital, School of Clinical Medicine, Shandong First Medical University","place":["Jinan, Shandong, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lin","family":"Bian","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Autoimmunology, Shandong Province University Clinical Immunology Translational Medicine Laboratory, The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital","place":["Jinan, Shandong, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiuhua","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Autoimmunology, Shandong Province University Clinical Immunology Translational Medicine Laboratory, The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital","place":["Jinan, Shandong, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tingting","family":"Zhai","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Autoimmunology, Shandong Province University Clinical Immunology Translational Medicine Laboratory, The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital","place":["Jinan, Shandong, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qing","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Autoimmunology, Shandong Province University Clinical Immunology Translational Medicine Laboratory, The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital","place":["Jinan, Shandong, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanfeng","family":"Hou","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Autoimmunology, Shandong Province University Clinical Immunology Translational Medicine Laboratory, The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital","place":["Jinan, Shandong, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,7,3]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000012989","article-title":"Relapsed\/refractory acquired thrombotic thrombocytopenic purpura in a patient with sj\u00f6gren syndrome: Case report and review of the literature","volume":"97","author":"Sun","year":"2018","journal-title":"Med (Baltimore)"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1007\/s12185-023-03657-0","article-title":"Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) in Japan 2023","volume":"118","author":"Matsumoto","year":"2023","journal-title":"Int J 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