{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,15]],"date-time":"2025-12-15T16:58:45Z","timestamp":1765817925955,"version":"3.48.0"},"reference-count":15,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2025,11,14]],"date-time":"2025-11-14T00:00:00Z","timestamp":1763078400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["bpspubs.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pharmacology Res &amp;amp; Perspec"],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Fibrodysplasia ossificans progressiva (FOP) is an ultra\u2010rare genetic disorder with inflammation\u2010related flare\u2010ups resulting in catastrophic heterotopic ossification (HO). Janus\u2010associated kinase (JAK) inhibitors may have had a blocking effect on bone formation in controlling FOP flare\u2010ups by blocking multiple inflammatory signaling pathways. Continuous JAK inhibitor tofacitinib treatment has shown preliminary safety and effect in preventing FOP flare\u2010ups. There is concern that the use of long\u2010term continuous JAK inhibitors might cause renal, hepatic, and hematological toxicity, as well as increased infections and cancers. We incorporated a six\u2010week ruxolitinib (another JAK inhibitor) regimen given intermittently for empirical use at the flare\u2010up onset in a teenager after she experienced three consecutive corticosteroid\u2010refractory severe lower limb FOP flare\u2010ups within 1\u2009year. The regimen proved well\u2010tolerated with efficacy in terms of blocking morbidity\u2010generating heterotopic ossification and extending the flare\u2010up\u2010free intervals to 5, 12, and 36\u2009months until subsequent flare\u2010ups, respectively, accompanied by a stable cumulative analog joint involvement scale (CAJIS) score for the subsequent 5\u2009years. The regimen appeared to inhibit new bone formation and may avoid long\u2010term use\u2010related toxicities in FOP patients.<\/jats:p>","DOI":"10.1002\/prp2.70193","type":"journal-article","created":{"date-parts":[[2025,11,14]],"date-time":"2025-11-14T11:20:51Z","timestamp":1763119251000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Intermittent and Short\u2010Term Empirical Ruxolitinib Regimen for Steroid\u2010Refractory Flareups of Fibrodysplasia Ossificans Progressiva"],"prefix":"10.1002","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0290-9886","authenticated-orcid":false,"given":"Rong\u2010Long","family":"Chen","sequence":"first","affiliation":[{"name":"Department of Pediatric Hematology and Oncology Koo Foundation Sun Yat\u2010Sen Cancer Center  Taipei Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tzu\u2010Hsien","family":"Yang","sequence":"additional","affiliation":[{"name":"Institute of Molecular Biology, Academia Sinica  Taipei Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yun\u2010Hsin","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine Koo Foundation Sun Yat\u2010Sen Cancer Center  Taipei Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jy\u2010juinn","family":"Shaw","sequence":"additional","affiliation":[{"name":"Institute of Molecular Biology, Academia Sinica  Taipei Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Liuh\u2010Yow","family":"Chen","sequence":"additional","affiliation":[{"name":"Institute of Molecular Biology, Academia Sinica  Taipei Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,11,14]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023\u2010021\u201001983\u20102"},{"key":"e_1_2_13_3_1","doi-asserted-by":"publisher","DOI":"10.3390\/biom14030357"},{"key":"e_1_2_13_4_1","first-page":"1","article-title":"The Medical Management of Fibrodysplasia Ossificans Progressiva: Current Treatment Considerations","volume":"3","author":"Kaplan F. S.","year":"2024","journal-title":"Proc International Clinical Council on FOP"},{"key":"e_1_2_13_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41591\u2010023\u201002561\u20108"},{"key":"e_1_2_13_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41409\u2010024\u201002252\u2010z"},{"key":"e_1_2_13_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2021014860"},{"key":"e_1_2_13_8_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12969\u2010023\u201000856\u20101"},{"key":"e_1_2_13_9_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.00377"},{"key":"e_1_2_13_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bone.2017.04.015"},{"key":"e_1_2_13_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/00003086\u2010199703000\u201000033"},{"key":"e_1_2_13_12_1","doi-asserted-by":"publisher","DOI":"10.15252\/embj.2020106317"},{"key":"e_1_2_13_13_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.24.00205"},{"key":"e_1_2_13_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkx1121"},{"key":"e_1_2_13_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/bph.16223"},{"key":"e_1_2_13_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/bph.16176"}],"container-title":["Pharmacology Research &amp; Perspectives"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/bpspubs.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/prp2.70193","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,15]],"date-time":"2025-12-15T16:56:07Z","timestamp":1765817767000},"score":1,"resource":{"primary":{"URL":"https:\/\/bpspubs.onlinelibrary.wiley.com\/doi\/10.1002\/prp2.70193"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,14]]},"references-count":15,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2025,12]]}},"alternative-id":["10.1002\/prp2.70193"],"URL":"https:\/\/doi.org\/10.1002\/prp2.70193","archive":["Portico"],"relation":{},"ISSN":["2052-1707","2052-1707"],"issn-type":[{"type":"print","value":"2052-1707"},{"type":"electronic","value":"2052-1707"}],"subject":[],"published":{"date-parts":[[2025,11,14]]},"assertion":[{"value":"2025-05-13","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-10-24","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-11-14","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70193"}}