{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T10:52:08Z","timestamp":1782125528572,"version":"3.54.5"},"reference-count":15,"publisher":"Walter de Gruyter GmbH","issue":"1","license":[{"start":{"date-parts":[[2025,1,1]],"date-time":"2025-01-01T00:00:00Z","timestamp":1735689600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Ministry of Science and Technology \u201c13th Five-Year\u201d National Key Research and Development Plan Key Project\u201d","award":["2019YFF0216502"],"award-info":[{"award-number":["2019YFF0216502"]}]},{"name":"Tianjin Medical Key Discipline (Specialty) Construction Project","award":["TJYXZDXK-071C"],"award-info":[{"award-number":["TJYXZDXK-071C"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,1,27]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome characterized by diverse etiologies and a high risk of misdiagnosis. Autoinflammatory disorders, such as adult-onset Still\u2019s disease (AOSD), are often underrecognized as contributing factors. Minimal change disease (MCD) represents an exceedingly rare renal complication in the context of HLH. This report describes the case of an adult female diagnosed with Epstein\u2013Barr virus (EBV)-associated HLH. Initial therapy with etoposide and dexamethasone yielded a partial response; however, disease relapse occurred, accompanied by the development of significant proteinuria. Renal biopsy confirmed a diagnosis of MCD. Administration of rituximab led to complete remission of the nephrotic syndrome, although HLH activity persisted. A subsequent skin biopsy revealed perivascular infiltration composed predominantly of lymphocytes and neutrophils. In combination with clinical features such as high-grade fever and rash, these findings supported a diagnosis of AOSD. Adjustment of the treatment regimen to include corticosteroids and methotrexate resulted in complete remission of HLH. This case underscores the importance of re-evaluating the underlying cause in patients with HLH who demonstrate an inadequate therapeutic response. MCD may represent a renal manifestation of immune dysregulation associated with HLH. A multidisciplinary approach is essential for improving diagnostic precision and optimizing treatment in complex presentations of\u00a0HLH.<\/jats:p>","DOI":"10.1515\/biol-2025-1228","type":"journal-article","created":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T16:27:55Z","timestamp":1767198475000},"source":"Crossref","is-referenced-by-count":0,"title":["Adult-onset Still\u2019s disease with hemophagocytic lymphohistiocytosis and minimal change disease"],"prefix":"10.1515","volume":"20","author":[{"given":"Dong-hua","family":"Guo","sequence":"first","affiliation":[{"name":"Department of Nephrology , Kidney Disease Medical Center, General Hospital, Tianjin Medical University, National Key Clinical Specialty, Tianjin Key Medical Discipline , Tianjin 300052 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Wei","sequence":"additional","affiliation":[{"name":"Department of Nephrology , Kidney Disease Medical Center, General Hospital, Tianjin Medical University, National Key Clinical Specialty, Tianjin Key Medical Discipline , Tianjin 300052 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun-ya","family":"Jia","sequence":"additional","affiliation":[{"name":"Department of Nephrology , Kidney Disease Medical Center, General Hospital, Tianjin Medical University, National Key Clinical Specialty, Tianjin Key Medical Discipline , Tianjin 300052 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"374","published-online":{"date-parts":[[2026,1,1]]},"reference":[{"key":"2026062210235400668_j_biol-2025-1228_ref_001","doi-asserted-by":"crossref","unstructured":"Naymagon, L, Roehrs, P, Hermiston, M, Connelly, J, Bednarski, J, Boelens, JJ, et al.. Perspectives on the current diagnostic and treatment paradigms in secondary hemophagocytic lymphohistiocytosis (HLH). Orphanet J Rare Dis 2025;20:200. https:\/\/doi.org\/10.1186\/s13023-025-03698-0.","DOI":"10.1186\/s13023-025-03698-0"},{"key":"2026062210235400668_j_biol-2025-1228_ref_002","doi-asserted-by":"crossref","unstructured":"Wang, R, Li, T, Ye, S, Tan, W, Zhao, C, Li, Y, et al.. Macrophage activation syndrome associated with adult-onset Still\u2019s disease: a multicenter retrospective analysis. Clin Rheumatol 2020;39:2379\u201386. https:\/\/doi.org\/10.1007\/s10067-020-04949-0.","DOI":"10.1007\/s10067-020-04949-0"},{"key":"2026062210235400668_j_biol-2025-1228_ref_003","doi-asserted-by":"crossref","unstructured":"Henter, JI. Hemophagocytic lymphohistiocytosis. N\u00a0Engl J Med 2025;392:584\u201398. https:\/\/doi.org\/10.1056\/nejmra2314005.","DOI":"10.1056\/NEJMra2314005"},{"key":"2026062210235400668_j_biol-2025-1228_ref_004","doi-asserted-by":"crossref","unstructured":"Todorovi\u0107, N, Ambrosio, MR, Amedei, A. Immune modulation by Epstein\u2013Barr virus lytic cycle: relevance and implication in oncogenesis. Pathogens 2024;13:876. https:\/\/doi.org\/10.3390\/pathogens13100876.","DOI":"10.3390\/pathogens13100876"},{"key":"2026062210235400668_j_biol-2025-1228_ref_005","doi-asserted-by":"crossref","unstructured":"Giacomelli, R, Ruscitti, P, Shoenfeld, Y. A comprehensive review on adult onset Still\u2019s disease. J\u00a0Autoimmun 2018;93:24\u201336. https:\/\/doi.org\/10.1016\/j.jaut.2018.07.018.","DOI":"10.1016\/j.jaut.2018.07.018"},{"key":"2026062210235400668_j_biol-2025-1228_ref_006","doi-asserted-by":"crossref","unstructured":"Lebrun, D, Mestrallet, S, Dehoux, M, Golmard, JL, Granger, B, Georgin-Lavialle, S, et al.. Validation of the Fautrel classification criteria for adult-onset Still\u2019s disease. Semin Arthritis Rheum 2018;47:578\u201385. https:\/\/doi.org\/10.1016\/j.semarthrit.2017.07.005.","DOI":"10.1016\/j.semarthrit.2017.07.005"},{"key":"2026062210235400668_j_biol-2025-1228_ref_007","doi-asserted-by":"crossref","unstructured":"Gerfaud-Valentin, M, Cottin, V, Jamilloux, Y, Hot, A, Gaillard-Coadon, A, Durieu, I, et al.. Parenchymal lung involvement in adult-onset Still disease: a STROBE-compliant case series and literature review. Medicine (Baltim) 2016;95:e4258. https:\/\/doi.org\/10.1097\/MD.0000000000004258.","DOI":"10.1097\/MD.0000000000004258"},{"key":"2026062210235400668_j_biol-2025-1228_ref_008","doi-asserted-by":"crossref","unstructured":"Xi, XT, Wang, MJ, Huang, RY, Ding, BH. Adult onset Still\u2019s disease accompanied by acute respiratory distress syndrome: a case report. 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Nat Rev Rheumatol 2018;14:603\u201318. https:\/\/doi.org\/10.1038\/s41584-018-0081-x.","DOI":"10.1038\/s41584-018-0081-x"},{"key":"2026062210235400668_j_biol-2025-1228_ref_012","doi-asserted-by":"crossref","unstructured":"Bae, CB, Jung, JY, Kim, HA, Suh, CH. Reactive hemophagocytic syndrome in adult-onset Still disease: clinical features, predictive factors, and prognosis in 21 patients. Medicine (Baltim) 2015;94:e451. https:\/\/doi.org\/10.1097\/MD.0000000000000451.","DOI":"10.1097\/MD.0000000000000451"},{"key":"2026062210235400668_j_biol-2025-1228_ref_013","doi-asserted-by":"crossref","unstructured":"Efthimiou, P, Kontzias, A, Hur, P, Rodha, K, Ramakrishna, GS, Nakasato, P. Adult-onset Still\u2019s disease in focus: clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies. 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J\u00a0Clin Pulm Med 2021;26:1084\u20137. https:\/\/doi.org\/10.3969\/j.issn.1009-6663.2021.07.025."}],"container-title":["Open Life Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.degruyterbrill.com\/document\/doi\/10.1515\/biol-2025-1228\/xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.degruyterbrill.com\/document\/doi\/10.1515\/biol-2025-1228\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T10:26:04Z","timestamp":1782123964000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.degruyterbrill.com\/document\/doi\/10.1515\/biol-2025-1228\/html"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1,1]]},"references-count":15,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,1,1]]},"published-print":{"date-parts":[[2025,1,27]]}},"alternative-id":["10.1515\/biol-2025-1228"],"URL":"https:\/\/doi.org\/10.1515\/biol-2025-1228","relation":{},"ISSN":["2391-5412"],"issn-type":[{"value":"2391-5412","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1,1]]},"article-number":"20251228"}}