{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T05:15:36Z","timestamp":1783401336520,"version":"3.54.6"},"reference-count":22,"publisher":"Springer Science and Business Media LLC","issue":"7","license":[{"start":{"date-parts":[[2026,5,23]],"date-time":"2026-05-23T00:00:00Z","timestamp":1779494400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,5,23]],"date-time":"2026-05-23T00:00:00Z","timestamp":1779494400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100005278","name":"University of Antioquia","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100005278","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Clin Rheumatol"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>TAFRO syndrome is a rare hyperinflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly. Its coexistence with Sj\u00f6gren\u2019s disease has only been reported in isolated cases, and both conditions are thought to share a pathogenic pathway mediated by the activation of the IL-6\/VEGF axis and the presence of anti-SSA\/Ro60 autoantibodies.\u00a0We present a 33-year-old woman with primary Sj\u00f6gren\u2019s disease who fulfilled the 2016 ACR\/EULAR criteria and developed anasarca, persistent fever, severe thrombocytopenia, and acute kidney injury due to thrombotic microangiopathy and tubulointerstitial nephritis. Despite receiving pulse therapy and high-dose glucocorticoids, she experienced progressive deterioration that required renal replacement therapy. Given the suspicion of TAFRO syndrome associated with a systemic inflammatory response, weekly subcutaneous tocilizumab was initiated, resulting in rapid improvement in her overall condition, resolution of anasarca, and complete recovery of renal function.\u00a0This case highlights the rare overlap between TAFRO syndrome and Sj\u00f6gren's disease, in which IL-6 activation plays a central role. The favorable response to IL-6 blockade emphasizes the importance of early recognition of this association and consideration of targeted therapies for refractory hyperinflammation.<\/jats:p>","DOI":"10.1007\/s10067-026-08075-1","type":"journal-article","created":{"date-parts":[[2026,5,23]],"date-time":"2026-05-23T02:11:54Z","timestamp":1779502314000},"page":"4667-4673","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["TAFRO syndrome complicating Sj\u00f6gren\u2019s disease: response to subcutaneous tocilizumab"],"prefix":"10.1007","volume":"45","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0929-7843","authenticated-orcid":false,"given":"N. A.","family":"Uribe-Ru\u00edz","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.","family":"Murillo\u2013Baquero","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. F.","family":"Vargas-Camacho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V.","family":"Santiago-Pacheco","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Taborda-Murillo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. H.","family":"Mu\u00f1oz-Vahos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L. A.","family":"Gonz\u00e1lez-Naranjo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. L.","family":"Vanegas-Garc\u00eda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,5,23]]},"reference":[{"issue":"1","key":"8075_CR1","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1002\/art.39859","volume":"69","author":"CH Shiboski","year":"2017","unstructured":"Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, Rasmussen A, Scofield H, Vitali C, Bowman SJ, Mariette X, International Sj\u00f6gren\u2019s Syndrome Criteria Working Group (2017) 2016 American College of Rheumatology\/European League against rheumatism classification criteria for primary Sj\u00f6gren\u2019s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Arthritis Rheumatol 69(1):35\u201345. https:\/\/doi.org\/10.1002\/art.39859","journal-title":"Arthritis Rheumatol"},{"key":"8075_CR2","unstructured":"Takai K, Nikkuni K, Shibuya H, Hashidate H (2010) Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly. Rinsho Ketsueki 51(5):320\u2013325"},{"issue":"10","key":"8075_CR3","doi-asserted-by":"publisher","first-page":"1241","DOI":"10.1002\/ajh.26292","volume":"96","author":"Y Nishimura","year":"2021","unstructured":"Nishimura Y, Fajgenbaum DC, Pierson SK, Iwaki N, Nishikori A, Kawano M, Nakamura N, Izutsu K, Takeuchi K, Nishimura MF, Maeda Y, Otsuka F, Yoshizaki K, Oksenhendler E, van Rhee F, Sato Y (2021) Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease. Am J Hematol 96(10):1241\u20131252. https:\/\/doi.org\/10.1002\/ajh.26292","journal-title":"Am J Hematol"},{"key":"8075_CR4","doi-asserted-by":"publisher","unstructured":"Masaki Y, Kawabata H, Takai K, Tsukamoto N, Fujimoto S, Ishigaki Y, Japanese TAFRO Syndrome Research Team et al (2020) 2019 updated diagnostic criteria and disease severity classification for TAFRO syndrome. Int J Hematol 111(1):155\u20138. https:\/\/doi.org\/10.1007\/s12185-019-02780-1","DOI":"10.1007\/s12185-019-02780-1"},{"issue":"2","key":"8075_CR5","doi-asserted-by":"publisher","first-page":"485","DOI":"10.1007\/s00277-022-04762-6","volume":"101","author":"Y Masaki","year":"2022","unstructured":"Masaki Y, Arita K, Sakai T, Takai K, Aoki S, Kawabata H (2022) Castleman disease and TAFRO syndrome. 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The information presented derives from routine clinical care and does not constitute a clinical trial. The patient provided written informed consent for the use of her anonymized clinical information for academic and publication purposes.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"The patient has provided her informed written consent for the publication of this case report, including any images or attached data, and guarantees anonymity and confidentiality.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"None.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Disclosures"}}]}}