{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T15:40:14Z","timestamp":1773934814004,"version":"3.50.1"},"reference-count":5,"publisher":"Danish Medical Association","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Ugeskr L\u00e6ger"],"abstract":"<jats:p>Haemophagocytic lymphohistiocytosis\/macrophage activation syndrome (HLH\/MAS) is a life-threatening condition characterised by systemic hyperinflammation and is often triggered by infections, malignancies, or autoimmune disease. The HScore is a diagnostic tool used to estimate the likelihood of HLH\/MAS based on clinical, biochemical, and histological findings. This case describes a patient with polymyositis developing MAS, indicated by an HScore of 219. He was treated with IV anakinra, steroids, and immunoglobulin, leading to significant improvement. This case emphasises the importance of early MAS recognition and timely intervention in critical conditions.<\/jats:p>","DOI":"10.61409\/v04250308","type":"journal-article","created":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T09:15:05Z","timestamp":1770887705000},"page":"1-3","source":"Crossref","is-referenced-by-count":0,"title":["Macrophage activation syndrome in a young patient with polymyositis"],"prefix":"10.61409","author":[{"given":"Stine","family":"Daugaard","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S\u00f8ren","family":"Jensen-Fangel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mads Nyhuus Bendix","family":"Rasch","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Morten Aagaard","family":"Nielsen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"40208","published-online":{"date-parts":[[2026,2,2]]},"reference":[{"key":"ref0","doi-asserted-by":"publisher","unstructured":"Shakoory B, Geerlinks A, Wilejto M, et al. The 2022 EULAR\/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis\/macrophage activation syndrome (HLH\/MAS). Arthritis Rheumatol. 2023;75(10):1714-1732. https:\/\/doi.org\/10.1002\/art.42636","DOI":"10.1002\/art.42636"},{"key":"ref1","doi-asserted-by":"publisher","unstructured":"Henter JI. Hemophagocytic lymphohistiocytosis. N Engl J Med. 2025;392(6):584-598. https:\/\/doi.org\/10.1056\/NEJMra2314005","DOI":"10.1056\/NEJMra2314005"},{"key":"ref2","doi-asserted-by":"publisher","unstructured":"Fardet L, Galicier L, Lambotte O, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014;66(9):2613-2620. https:\/\/doi.org\/10.1002\/art.38690","DOI":"10.1002\/art.38690"},{"key":"ref3","doi-asserted-by":"publisher","unstructured":"Bindoli S, De Matteis A, Mitrovic S, et al. Efficacy and safety of therapies for Still's disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR\/PReS guidelines for the management of Still's disease. Ann Rheum Dis. 2024;83(12):1731-1747. https:\/\/doi.org\/10.1136\/ard-2024-225854","DOI":"10.1136\/ard-2024-225854"},{"key":"ref4","doi-asserted-by":"publisher","unstructured":"Charlesworth JEG, Kavirayani A. Intravenous anakinra for the treatment of haemophagocytic lymphohistiocytosis\/macrophage activation syndrome: a systematic review. Eur J Haematol. 2023;111(3):458-476. https:\/\/doi.org\/10.1111\/ejh.14029","DOI":"10.1111\/ejh.14029"}],"container-title":["Ugeskrift for L\u00e6ger"],"original-title":[],"deposited":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T13:30:51Z","timestamp":1773927051000},"score":1,"resource":{"primary":{"URL":"https:\/\/ugeskriftet.dk\/videnskab\/V04250308"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,2]]},"references-count":5,"URL":"https:\/\/doi.org\/10.61409\/v04250308","relation":{},"ISSN":["0041-5782","1603-6824"],"issn-type":[{"value":"0041-5782","type":"print"},{"value":"1603-6824","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,2]]}}}