{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T17:13:18Z","timestamp":1793293998639,"version":"4.5.0"},"reference-count":14,"publisher":"BMJ","issue":"2","license":[{"start":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T00:00:00Z","timestamp":1779321600000},"content-version":"unspecified","delay-in-days":50,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["RMD Open"],"accepted":{"date-parts":[[2026,4,8]]},"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Thrombocytopaenia, anasarca, fever, reticulin fibrosis, renal dysfunction and organomegaly (TAFRO) syndrome has been reported to be associated with anti-SS-A antibodies, which activate type I interferon (IFN) signalling. We propose a subclassification of TAFRO syndrome based on the anti-SS-A antibody status, with the aim of identifying effective treatment strategies.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We reviewed TAFRO syndrome cases from the PubMed\/MEDLINE databases from January 1977 to October 2024. The information on concomitant autoimmune diseases, the presence of anti-SS-A antibodies, serum IgG levels, treatment courses and clinical outcomes was collected and retrospectively analysed. Patients were stratified according to anti-SS-A antibody status, and response to tocilizumab (TCZ) or rituximab (RTX) was evaluated.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Among 212 cases, 27 had concomitant autoimmune diseases, with Sj\u00f6gren\u2019s disease (SjD) being the most common. Anti-SS-A antibody positivity was observed in 37 cases, of which 12 fulfilled the classification criteria for SjD. RTX was significantly more effective in anti-SS-A antibody-positive patients than in antibody-negative patients (88.9% vs 16.7%, p=0.011), whereas therapeutic response to TCZ was not affected by anti-SS-A antibody status. Serum IgG levels were significantly higher in anti-SS-A antibody-positive patients than in antibody-negative patients (p=0.020).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>TAFRO syndrome shows clinical heterogeneity and anti-SS-A antibody status may represent one potential factor associated with treatment response. Further studies are needed to confirm these findings and clarify the underlying mechanisms.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/rmdopen-2025-006547","type":"journal-article","created":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T15:35:45Z","timestamp":1779377745000},"page":"e006547","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":2,"title":["Potential stratification of TAFRO syndrome by anti-SS-A antibodies status and therapeutic response to rituximab"],"prefix":"10.1136","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-0569-0433","authenticated-orcid":false,"given":"Tomohiro","family":"Suzuki","sequence":"first","affiliation":[{"name":"Department of Allergy and Rheumatology, The University of Tokyo Hospital, Bunkyo, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saeko","family":"Yamada","sequence":"additional","affiliation":[{"name":"Department of Allergy and Rheumatology, The University of Tokyo Hospital, Bunkyo, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kanto","family":"Toriumi","sequence":"additional","affiliation":[{"name":"Department of Allergy and Rheumatology, The University of Tokyo Hospital, Bunkyo, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masanori","family":"Kono","sequence":"additional","affiliation":[{"name":"Department of Allergy and Rheumatology, The University of Tokyo Hospital, Bunkyo, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haruka","family":"Tsuchiya","sequence":"additional","affiliation":[{"name":"Department of Allergy and Rheumatology, The University of Tokyo Hospital, Bunkyo, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7276-5254","authenticated-orcid":false,"given":"Keishi","family":"Fujio","sequence":"additional","affiliation":[{"name":"Department of Allergy and Rheumatology, The University of Tokyo Hospital, Bunkyo, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2026,5,21]]},"reference":[{"key":"2026052108352790000_12.2.e006547.1","first-page":"320","article-title":"Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly","volume":"51","author":"Takai","year":"2010","journal-title":"Rinsho Ketsueki"},{"key":"2026052108352790000_12.2.e006547.2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.24242"},{"key":"2026052108352790000_12.2.e006547.3","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1007\/s00277-022-04762-6","article-title":"Castleman disease and TAFRO syndrome","volume":"101","author":"Masaki","year":"2022","journal-title":"Ann Hematol"},{"key":"2026052108352790000_12.2.e006547.4","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1007\/s12185-019-02780-1","article-title":"2019 Updated diagnostic criteria and disease severity classification for TAFRO syndrome","volume":"111","author":"Masaki","year":"2020","journal-title":"Int J Hematol"},{"key":"2026052108352790000_12.2.e006547.5","doi-asserted-by":"crossref","DOI":"10.1038\/s41598-024-53413-5","article-title":"TAFRO syndrome is associated with anti-SSA\/Ro60 antibodies, in contrast to idiopathic castleman disease","volume":"14","author":"Shirakashi","year":"2024","journal-title":"Sci Rep"},{"key":"2026052108352790000_12.2.e006547.6","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197907053010102"},{"key":"2026052108352790000_12.2.e006547.7","doi-asserted-by":"publisher","DOI":"10.1038\/nrrheum.2018.31"},{"key":"2026052108352790000_12.2.e006547.8","doi-asserted-by":"crossref","first-page":"103137","DOI":"10.1016\/j.autrev.2022.103137","article-title":"TAFRO syndrome: a severe manifestation of Sj\u00f6gren\u2019s syndrome? A systematic review","volume":"21","author":"Grange","year":"2022","journal-title":"Autoimmun Rev"},{"key":"2026052108352790000_12.2.e006547.9","doi-asserted-by":"crossref","first-page":"103438","DOI":"10.1016\/j.jaut.2025.103438","article-title":"Interferon signatures fuel B cell hyperactivity and plasmablast expansion in systemic lupus erythematosus","volume":"154","author":"van Dooren","year":"2025","journal-title":"J Autoimmun"},{"key":"2026052108352790000_12.2.e006547.10","doi-asserted-by":"publisher","DOI":"10.1007\/s10165-004-0338-x"},{"key":"2026052108352790000_12.2.e006547.11","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1002\/art.39859","article-title":"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","volume":"69","author":"Shiboski","year":"2017","journal-title":"Arthritis & Rheumatology"},{"key":"2026052108352790000_12.2.e006547.12","doi-asserted-by":"crossref","DOI":"10.1172\/jci.insight.135031","article-title":"Type I IFN response associated with mTOR activation in the TAFRO subtype of idiopathic multicentric Castleman disease","volume":"5","author":"Pai","year":"2020","journal-title":"JCI Insight"},{"key":"2026052108352790000_12.2.e006547.13","doi-asserted-by":"crossref","first-page":"100473","DOI":"10.1016\/j.xgen.2023.100473","article-title":"Single-cell transcriptome landscape of circulating CD4+ T cell populations in autoimmune diseases","volume":"4","author":"Yasumizu","year":"2024","journal-title":"Cell Genomics"},{"key":"2026052108352790000_12.2.e006547.14","doi-asserted-by":"crossref","DOI":"10.1093\/ckj\/sfae110","article-title":"Long-term clinicopathological characteristics of TAFRO syndrome and its relapse: a case series study","volume":"17","author":"Yoshimura","year":"2024","journal-title":"Clin Kidney J"}],"container-title":["RMD Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/rmdopen-2025-006547","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T15:35:47Z","timestamp":1779377747000},"score":1,"resource":{"primary":{"URL":"https:\/\/rmdopen.bmj.com\/lookup\/doi\/10.1136\/rmdopen-2025-006547"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4]]},"references-count":14,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2026,5,21]]},"published-print":{"date-parts":[[2026,4]]}},"alternative-id":["10.1136\/rmdopen-2025-006547"],"URL":"https:\/\/doi.org\/10.1136\/rmdopen-2025-006547","relation":{},"ISSN":["2056-5933"],"issn-type":[{"value":"2056-5933","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4]]}}}