{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T06:05:04Z","timestamp":1780553104995,"version":"3.54.1"},"reference-count":23,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T00:00:00Z","timestamp":1780531200000},"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. Pediatr."],"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP) and juvenile idiopathic arthritis (JIA) both feature joint effusions. This study aimed to identify preliminary MSUS-based criteria that may help discriminate children with CACP from those with polyarticular JIA.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A retrospective multicentre analysis of MSUS images from children with genetically confirmed CACP or a diagnosis of active polyarticular JIA was performed. Joint and tendon findings were evaluated using qualitative B-mode (BM) and Doppler-mode (DM) assessment and graded semi-quantitatively (0\u20133) according to OMERACT definitions.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      All four children with CACP showed symmetrical joint involvement, with effusion present in 46\/46 scanned joints and no intrasynovial hypervascularity (median DM\u2009=\u20090). In the JIA cohort (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20096) 16\/42 joints had asymmetric effusions, of which 56% showed intrasynovial hypervascularity (median DM\u2009=\u20091). All 16\/16 scanned tendons in the CACP group showed no DM signal, whereas hypervascularity was observed in 7\/8 affected tendons in the JIA cohort. Hyperechoic foci, suggestive of increased synovial fluid viscosity, were present in 18\/46 (39%) affected joints in the CACP group compared to 1\/16 (6%) in the JIA cohort.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>CACP is characterised by symmetrical large-joint effusions with hyperechoic foci and absence of intrasynovial DM signal, in contrast to inflammatory findings in JIA. These features may support early differentiation of CACP from JIA and therefore prompt earlier specific diagnostics, including genetic testing.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fped.2026.1829170","type":"journal-article","created":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T05:40:56Z","timestamp":1780551656000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Musculoskeletal ultrasound discriminating camptodactyly-arthropathy-coxa vara-pericarditis syndrome and juvenile idiopathic arthritis"],"prefix":"10.3389","volume":"14","author":[{"given":"Hatice","family":"Adig\u00fczel Dundar","sequence":"first","affiliation":[{"name":"Clinic of Pediatric and Adolescent Rheumatology, St.-Josef-Stift Sendenhorst, Northwest Germany Center for Rheumatology","place":["Sendenhorst, Germany"]},{"name":"Behcet Uz Pediatric Diseases and Surgery Training and Research Hospital, Clinic of Pediatric Rheumatology, University of Health Sciences","place":["Izmir, T\u00fcrkiye"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Faekah","family":"Gohar","sequence":"additional","affiliation":[{"name":"Clinic of Pediatric and Adolescent Rheumatology, St.-Josef-Stift Sendenhorst, Northwest Germany Center for Rheumatology","place":["Sendenhorst, Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Windschall","sequence":"additional","affiliation":[{"name":"Clinic of Pediatric and Adolescent Rheumatology, St.-Josef-Stift Sendenhorst, Northwest Germany Center for Rheumatology","place":["Sendenhorst, Germany"]},{"name":"Medical Faculty, Martin Luther University Halle-Wittenberg","place":["Halle, Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,6,4]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"535","DOI":"10.1006\/bbrc.1998.0104","article-title":"Articular cartilage superficial zone protein (SZP) is homologous to megakaryocyte stimulating factor precursor and is a multifunctional proteoglycan with potential growth-promoting, cytoprotective, and lubricating properties in cartilage metabolism","volume":"254","author":"Flannery","year":"1999","journal-title":"Biochem Biophys Res Commun"},{"key":"B2","doi-asserted-by":"publisher","first-page":"622","DOI":"10.1172\/JCI22263","article-title":"The secreted glycoprotein lubricin protects cartilage surfaces and inhibits synovial cell overgrowth","volume":"115","author":"Rhee","year":"2005","journal-title":"J Clin Invest"},{"key":"B3","doi-asserted-by":"publisher","first-page":"230","DOI":"10.1002\/mgg3.364","article-title":"Genotype-phenotype investigation of 35 patients from 11 unrelated families with camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome","volume":"6","author":"Yilmaz","year":"2018","journal-title":"Mol Genet Genomic Med"},{"key":"B4","doi-asserted-by":"publisher","first-page":"24","DOI":"10.4103\/2156-7514.114211","article-title":"Camptodactyly-arthropathy-coxa vara-pericarditis syndrome: important differential for juvenile idiopathic arthritis","volume":"3","author":"Kakkar","year":"2013","journal-title":"J Clin Imaging Sci"},{"key":"B5","doi-asserted-by":"publisher","first-page":"522","DOI":"10.2214\/ajr.185.2.01850522","article-title":"Camptodactyly-arthropathy-coxa vara-pericarditis syndrome versus juvenile idiopathic arthropathy","volume":"185","author":"Offiah","year":"2005","journal-title":"AJR Am J Roentgenol"},{"key":"B6","doi-asserted-by":"publisher","first-page":"101570","DOI":"10.1016\/j.berh.2020.101570","article-title":"Ultrasound imaging in paediatric rheumatology","volume":"34","author":"Windschall","year":"2020","journal-title":"Best Pract Res Clin Rheumatol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"2046","DOI":"10.1093\/rheumatology\/keab004","article-title":"The new role of musculoskeletal ultrasound in the treat-to-target management of juvenile idiopathic arthritis","volume":"60","author":"Gohar","year":"2021","journal-title":"Rheumatology (Oxford)"},{"key":"B8","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1097\/MCD.0000000000000500","article-title":"Thirteen Indians with camptodactyly-arthropathy-coxa vara-pericarditis syndrome","volume":"33","author":"Singh","year":"2024","journal-title":"Clin Dysmorphol"},{"key":"B9","doi-asserted-by":"publisher","first-page":"459","DOI":"10.11152\/mu-4452","article-title":"Ultrasound\u2019s role in differentiating camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome from inflammatory arthritis in children. 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