{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,28]],"date-time":"2026-01-28T04:35:30Z","timestamp":1769574930203,"version":"3.49.0"},"reference-count":19,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,1,27]],"date-time":"2026-01-27T00:00:00Z","timestamp":1769472000000},"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. Med."],"abstract":"<jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Hemophilia A (HA) and hemophilia B (HB) are X-linked-bleeding disorders caused by deficiency of clotting factors VIII and IX, while von Willebrand disease (vWD) type 3 involves the lack of von Willebrand factor and FVIII. Chronic joint damage from recurrent bleeding is a serious complication.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>The aim was to investigate the association of prophylactical treatment, severity of the disease and joint outcome.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>In this retrospective, single-center study we evaluated joint health in 41 patients with HA, HB, and vWD type 3 who visited our outpatient clinic since 2000 using Magnetic resonance imaging (MRI) and applied the International Prophylaxis Study Group (IPSG) score. A total of 246 MRI images (knees, elbows, ankles) were analyzed in relation to disease severity, genetics, inhibitor-formation, and therapy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Of 41 patients, 28 (68%) had severe HA or HB, 10 (24%) moderate, one (2%) mild, and two (5%) were vWD patients. 19 patients with severe HA\/HB received primary prophylaxis. Inhibitors developed in 7 patients (17%), most of them had loss-of-function mutations. We observed hemophilic arthropathy in 7\/39 (18%) hemophilia patients (all with severe HA\/HB). Only one of the 19 patients receiving early prophylaxis developed arthropathy, in the context of inhibitor development. Minor changes (IPSG score 1\u20135) were observed in 20% of joints while 74% of joints showed no alterations (IPSG score 0). Only 6% of joints showed hemophilic arthropathy (IPSG score \u2265 8) with ankle joints most frequently affected (10%). Among vWD-patients, one exhibited minor changes; the other had no detectable joint damage despite vWF-inhibitor presence.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>This study shows that the IPSG score is a suitable tool for assessing joint health in patients with hemophilia and vWD. Reduced joint damage was associated with early diagnosis, consistent prophylaxis, and therapy adherence.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fmed.2025.1668012","type":"journal-article","created":{"date-parts":[[2026,1,27]],"date-time":"2026-01-27T15:47:40Z","timestamp":1769528860000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Impact of prophylaxis, inhibitors, and genetics on joint outcomes according to the IPSG-MRI score in hemophilia A, B and vWD type 3"],"prefix":"10.3389","volume":"12","author":[{"given":"Anna","family":"Seeliger","sequence":"first","affiliation":[{"name":"Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, Faculty of Medicine, Children\u2019s Hospital, Medical Center, University of Freiburg","place":["Freiburg, 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