{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,26]],"date-time":"2026-01-26T04:56:42Z","timestamp":1769403402555,"version":"3.49.0"},"reference-count":34,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,1,6]],"date-time":"2026-01-06T00:00:00Z","timestamp":1767657600000},"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:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>TAFRO syndrome is a rare and severe variant of idiopathic multicentric Castleman disease (iMCD). The name-giving presentation is a combination of thrombocytopenia, anasarca, fever, reticulin fibrosis\/renal dysfunction, and organomegaly. The disease\u2019s complex but unspecific presentation shows overlapping features with hyperinflammation syndromes of infectious, malignant, or autoimmune origin. Here, we present a case of a young patient with iMCD TAFRO refractory to targeted IL-6 inhibition.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case description<\/jats:title>\n                    <jats:p>A 21-year-old previously healthy male developed progressive systemic inflammation with fever, persistent generalized lymphadenopathy, bicytopenia, polyserositis, and renal failure. Infectious diseases and oncological and immunological workup within the first 6 months after symptom onset yielded an unclear hyperinflammatory syndrome. Subsequently, the patient was treated with IL-1 inhibition resulting in partial symptom relief. However, after a flare, the repeated lymph node histopathology showed immunohistochemical features of iMCD and the diagnosis iMCD TAFRO was established. The patient was then additionally treated with IL-6 inhibition. An attempt to switch to monotherapy with IL-6 inhibition resulted in another flare-up of the disease. This demonstrated the need for continued combined IL-6 and IL-1 inhibition. Under this combination therapy, the patient showed complete and stable remission, which persisted even after 12 months of follow-up.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case highlights the diagnostic and therapeutic challenges posed by iMCD TAFRO. In cases with refractory disease despite targeted IL-6 inhibition, additional IL-1 inhibition might pose a further treatment option.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fimmu.2025.1729525","type":"journal-article","created":{"date-parts":[[2026,1,6]],"date-time":"2026-01-06T06:35:20Z","timestamp":1767681320000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["TAFRO syndrome requiring combined IL 6 and IL 1 inhibition: a case report"],"prefix":"10.3389","volume":"16","author":[{"given":"Ahmad Wael","family":"Sultan","sequence":"first","affiliation":[{"name":"Department of Gastroenterology, Infectious Diseases and Rheumatology, Charit\u00e9 Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin and Humboldt Universit\u00e4t zu Berlin and the Berlin Institute of Health (BIH), BIH Center for Regenerative Therapies","place":["Berlin, Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eva","family":"Grie\u00dfhammer","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Intensive Care Medicine, Charit\u00e9 Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin and Humboldt Universit\u00e4t zu Berlin and the Berlin Institute of Health (BIH), BIH Center for Regenerative Therapies","place":["Berlin, Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carl","family":"Hinrichs","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Intensive Care Medicine, Charit\u00e9 Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin and Humboldt Universit\u00e4t zu Berlin and the Berlin Institute of Health (BIH), BIH Center for Regenerative Therapies","place":["Berlin, Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lars","family":"Trenkmann","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Intensive Care Medicine, Charit\u00e9 Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin and Humboldt Universit\u00e4t zu Berlin and the Berlin Institute of Health (BIH), BIH Center for Regenerative Therapies","place":["Berlin, Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ilske","family":"Oschlies","sequence":"additional","affiliation":[{"name":"Department of Hematopathology and Lymph Node Registry (Division of Pathology), Universit\u00e4tsklinikum Schleswig-Holstein","place":["Kiel, Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Leif Gunnar","family":"Hanitsch","sequence":"additional","affiliation":[{"name":"Institute of Medical Immunology, Charit\u00e9-Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin and Humboldt Universit\u00e4t zu Berlin and the Berlin Institute of Health (BIH), BIH Center for Regenerative Therapies","place":["Berlin, Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hildrun","family":"Haibel","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, Infectious Diseases and Rheumatology, Charit\u00e9 Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin and Humboldt Universit\u00e4t zu Berlin and the Berlin Institute of Health (BIH), BIH Center for Regenerative Therapies","place":["Berlin, Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Schneider","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, Infectious Diseases and Rheumatology, Charit\u00e9 Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin and Humboldt Universit\u00e4t zu Berlin and the Berlin Institute 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