{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T05:01:04Z","timestamp":1788930064174,"version":"build-2803163510"},"reference-count":31,"publisher":"Oxford University Press (OUP)","issue":"8","license":[{"start":{"date-parts":[[2019,3,1]],"date-time":"2019-03-01T00:00:00Z","timestamp":1551398400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["asbmr.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["JBMR Plus"],"published-print":{"date-parts":[[2019,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:label\/><jats:p>Fibrodysplasia ossificans progressiva (FOP) is an ultra\u2010rare, inherited, connective tissue disease with \u223c800 documented cases worldwide. The principal pathological feature of FOP is the transition of skeletal muscle, tendons, ligaments, and fascia into cartilage and bone. This heterotopic ossification (HO) is often preceded by painful soft tissue swellings or flare\u2010ups that may last several months. For many individuals, experiencing a flare\u2010up may represent a worsening of their condition and contribute to feelings of anxiety or suppressed affect, both of which are well\u2010recognized to exacerbate pain perception. To date, much remains unknown regarding the dynamics of pain and emotional health in FOP during flare\u2010up and also quiescent, non\u2013flare\u2010up disease phases. In order to elucidate the occurrence and effect of pain in FOP, this study analyzed Patient\u2010Reported Outcomes Measurement Information System\u2013based questionnaires completed by 99 patients participating in the international FOP Registry over a 30\u2010month period. We observed that although moderate to severe pain (\u22654, 0 to 10 pain scale) was commonly associated with flare\u2010ups (56% to 67%), surprisingly, 30% to 55% of patients experienced similar pain levels during non\u2013flare\u2010up states. In those patients reporting pain levels of \u22654, 45% to 74% of patients report experiencing anxiety, depression, or irritability, with 36% to 48% reporting emotional problems during no to mild pain states. Furthermore, independent of the flare\u2010up status, the severity of pain in FOP patients was found to be significantly anti\u2010correlated with emotional health, physical health, and overall quality\u2010of\u2010life. These findings strongly suggest the need for an improved understanding of pain and emotional health in FOP during flare\u2010up and quiescent periods. \u00a9 2019 The Authors.<jats:italic>JBMR Plus<\/jats:italic>published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jbm4.10181","type":"journal-article","created":{"date-parts":[[2019,2,13]],"date-time":"2019-02-13T19:04:59Z","timestamp":1550084699000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Longitudinal Evaluation of Pain, Flare\u2010Up, and Emotional Health in Fibrodysplasia Ossificans Progressiva: Analyses of the International FOP Registry"],"prefix":"10.1093","volume":"3","author":[{"given":"Ke","family":"Peng","sequence":"first","affiliation":[{"name":"Center for Pain and the Brain Department of Anesthesiology Critical Care and Pain Medicine Boston Children's Hospital Harvard Medical School Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kin","family":"Cheung","sequence":"additional","affiliation":[{"name":"BioSAS Consulting, Inc. Wellesley MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arielle","family":"Lee","sequence":"additional","affiliation":[{"name":"Center for Pain and the Brain Department of Anesthesiology Critical Care and Pain Medicine Boston Children's Hospital Harvard Medical School Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Sieberg","sequence":"additional","affiliation":[{"name":"Center for Pain and the Brain Department of Anesthesiology Critical Care and Pain Medicine Boston Children's Hospital Harvard Medical School Boston MA USA"},{"name":"Biobehavioral Pediatric Pain Laboratory Department of Psychiatry Boston Children's Hospital Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Borsook","sequence":"additional","affiliation":[{"name":"Center for Pain and the Brain Department of Anesthesiology Critical Care and Pain Medicine Boston Children's Hospital Harvard Medical School Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaymin","family":"Upadhyay","sequence":"additional","affiliation":[{"name":"Center for Pain and the Brain Department of Anesthesiology Critical Care and Pain Medicine Boston Children's Hospital Harvard Medical School Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2019,3]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-017-0674-5"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1783"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1159\/000365770"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.aac4358"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/jbmr.2728"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/jbmr.3235"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bone.2017.08.012"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bone.2017.08.009"},{"issue":"8","key":"e_1_2_7_10_1","first-page":"RD01","article-title":"Clinical and genetic analysis of fibrodysplasia ossificans progressiva: a case report and literature review","volume":"9","author":"Lakkireddy M","year":"2015","journal-title":"J Clin Diagn Res"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-012-6562-y"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bone.2017.08.032"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.mlr.0000258615.42478.55"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00198-007-0425-x"},{"key":"e_1_2_7_15_1","doi-asserted-by":"crossref","unstructured":"ArnstadED RypdalV PeltoniemiS et al. Early self\u2010reported pain in juvenile idiopathic arthritis (JIA) is related to long\u2010term outcomes. Results from the Nordic JIA cohort study. Arthritis Care Res (Hoboken). Forthcoming. Epub2018Jul 28. DOI:10.1002\/acr.23715.","DOI":"10.1002\/acr.23715"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/00002508-200112000-00009"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1155\/2012\/420676"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpain.2003.08.001"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1097\/j.pain.0000000000001143"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2004-0682"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/ejp.836"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bone.2017.08.011"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1002\/jbm4.10008"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00296\u2010018\u20104179\u2010x"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1185\/03007995.2016.1157460"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.mlr.0000250483.85507.04"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jval.2016.02.009"},{"issue":"10","key":"e_1_2_7_28_1","doi-asserted-by":"crossref","first-page":"1625","DOI":"10.1002\/acr.22025","article-title":"Validity and reliability of patient\u2010reported outcomes measurement information system instruments in osteoarthritis","volume":"65","author":"Broderick JE","year":"2013","journal-title":"Arthritis Care Res"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3182872e80"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jclinepi.2015.08.038"},{"issue":"2","key":"e_1_2_7_31_1","first-page":"314","article-title":"Performance of a Patient Reported Outcomes Measurement Information System (PROMIS) short form in older adults with chronic musculoskeletal pain","volume":"17","author":"Deyo RA","year":"2016","journal-title":"Pain Med"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1097\/j.pain.0000000000000378"}],"container-title":["JBMR Plus"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fjbm4.10181","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jbm4.10181","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/jbm4.10181","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jbm4.10181","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,7,14]],"date-time":"2024-07-14T19:31:24Z","timestamp":1720985484000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jbmrplus\/article\/7479029"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,3]]},"references-count":31,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2019,8]]}},"alternative-id":["10.1002\/jbm4.10181"],"URL":"https:\/\/doi.org\/10.1002\/jbm4.10181","archive":["Portico"],"relation":{},"ISSN":["2473-4039","2473-4039"],"issn-type":[{"value":"2473-4039","type":"print"},{"value":"2473-4039","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,3]]},"assertion":[{"value":"2018-11-26","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-01-30","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-03-01","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e10181"}}