{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T23:46:34Z","timestamp":1790725594522,"version":"4.1.0"},"reference-count":18,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2022,9,5]],"date-time":"2022-09-05T00:00:00Z","timestamp":1662336000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,9,5]],"date-time":"2022-09-05T00:00:00Z","timestamp":1662336000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Rheumatol"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Sj\u00f6gren disease in children and adolescents (pedSD) presents differently than adult disease. Diagnosis and classification are controversial, optimal treatment is unknown and outcomes are poorly understood. Here, we describe the current perspectives of pediatric rheumatologists on diagnosis, treatment, and outcomes of pedSD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A voluntary, 17-question survey was distributed to providers in the Childhood Arthritis and Rheumatology Research Alliance and\/or the American College of Rheumatology Childhood Sj\u00f6gren\u2019s Study Group at the 2020 Convergence Virtual Conference. Findings are reported using descriptive statistics and chi-square testing.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Of 465 eligible providers, 157 (34%) responded with 135 (29%) completing the survey. The majority (85%) saw five or fewer patients with pedSD in the past year. Parotitis, dry eye and\/or dry mouth, and constitutional symptoms were among the most specific and common clinical features. Most providers (77%) used clinical judgment guided by adult criteria for diagnosis. The vast majority (86\u201399%) of survey participants indicated routine use of serologic testing, while salivary gland ultrasound, minor salivary gland biopsy and other diagnostic tests were less often used. The most commonly prescribed systemic immunomodulators were hydroxychloroquine, corticosteroids, methotrexate, rituximab, and mycophenolate. Seven providers reported malignancy in a patient with pedSD, including one death.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Pediatric rheumatologists diagnose and treat pedSD; however, most only see a few patients per year and rely on clinical judgment and laboratory testing for diagnosis. Treatment frequently includes systemic immunomodulators and malignancies are reported. More studies are needed to better understand natural history, risk factors, and the impact of interventions on outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s12969-022-00740-4","type":"journal-article","created":{"date-parts":[[2022,9,5]],"date-time":"2022-09-05T14:02:34Z","timestamp":1662386554000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Pediatric rheumatologists\u2019 perspectives on diagnosis, treatment, and outcomes of Sj\u00f6gren disease in children and adolescents"],"prefix":"10.1186","volume":"20","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8009-8209","authenticated-orcid":false,"given":"Rachel L.","family":"Randell","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara M.","family":"Stern","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heather","family":"Van Mater","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura E.","family":"Schanberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott M.","family":"Lieberman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew L.","family":"Basiaga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"CARRA Sj\u00f6gren Workgroup","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"CARRA Investigators","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,9,5]]},"reference":[{"issue":"7","key":"740_CR1","doi-asserted-by":"publisher","first-page":"3144","DOI":"10.1093\/rheumatology\/keaa757","volume":"60","author":"ML Basiaga","year":"2021","unstructured":"Basiaga ML, Stern SM, Mehta JJ, Edens C, Randell RL, Pomorska A, et al. Childhood Sjogren syndrome: features of an international cohort and application of the 2016 ACR\/EULAR classification criteria. Rheumatology (Oxford). 2021;60(7):3144\u201355.","journal-title":"Rheumatology (Oxford)"},{"issue":"4","key":"740_CR2","doi-asserted-by":"publisher","first-page":"903","DOI":"10.1016\/j.semarthrit.2020.11.004","volume":"51","author":"A Marino","year":"2021","unstructured":"Marino A, Romano M, Giani T, Gaggiano C, Costi S, Singh R, et al. Childhood Sjogren\u2019s syndrome: An Italian case series and a literature review-based cohort. Semin Arthritis Rheum. 2021;51(4):903\u201310.","journal-title":"Semin Arthritis Rheum"},{"issue":"10","key":"740_CR3","doi-asserted-by":"publisher","first-page":"4558","DOI":"10.1093\/rheumatology\/keab032","volume":"60","author":"M Ramos-Casals","year":"2021","unstructured":"Ramos-Casals M, Acar-Denizli N, Vissink A, Brito-Zeron P, Li X, Carubbi F, et al. Childhood-onset of primary Sjogren\u2019s syndrome: phenotypic characterization at diagnosis of 158 children. Rheumatology (Oxford). 2021;60(10):4558\u201367.","journal-title":"Rheumatology (Oxford)"},{"issue":"4","key":"740_CR4","doi-asserted-by":"publisher","first-page":"707","DOI":"10.1016\/j.rdc.2021.07.008","volume":"47","author":"RL Randell","year":"2021","unstructured":"Randell RL, Lieberman SM. Unique Aspects of Pediatric Sjogren Disease. Rheum Dis Clin North Am. 2021;47(4):707\u201323.","journal-title":"Rheum Dis Clin North Am"},{"issue":"1","key":"740_CR5","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1136\/annrheumdis-2016-210571","volume":"76","author":"CH Shiboski","year":"2017","unstructured":"Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, et al. 2016 American College of Rheumatology\/European League Against Rheumatism classification criteria for primary Sjogren\u2019s syndrome: A consensus and data-driven methodology involving three international patient cohorts. Ann Rheum Dis. 2017;76(1):9\u201316.","journal-title":"Ann Rheum Dis"},{"key":"740_CR6","unstructured":"Flores-Chavez A, Kostov B, Solans R, Fraile G, Maure B, Feijoo-Masso C, et al. Severe, life-threatening phenotype of primary Sjogren's syndrome: clinical characterisation and outcomes in 1580 patients (GEAS-SS Registry). Clin Exp Rheumatol. 2018;36 Suppl 112(3):121\u20139."},{"issue":"25","key":"740_CR7","doi-asserted-by":"publisher","first-page":"e3766","DOI":"10.1097\/MD.0000000000003766","volume":"95","author":"S Fragkioudaki","year":"2016","unstructured":"Fragkioudaki S, Mavragani CP, Moutsopoulos HM. Predicting the risk for lymphoma development in Sjogren syndrome: An easy tool for clinical use. Medicine (Baltimore). 2016;95(25):e3766.","journal-title":"Medicine (Baltimore)"},{"issue":"7","key":"740_CR8","doi-asserted-by":"publisher","first-page":"1163","DOI":"10.1093\/rheumatology\/key392","volume":"58","author":"C Anquetil","year":"2019","unstructured":"Anquetil C, Hachulla E, Machuron F, Mariette X, Le Guern V, Vittecoq O, et al. Is early-onset primary Sjogren\u2019s syndrome a worse prognosis form of the disease? Rheumatology (Oxford). 2019;58(7):1163\u20137.","journal-title":"Rheumatology (Oxford)"},{"issue":"8","key":"740_CR9","doi-asserted-by":"publisher","first-page":"1228","DOI":"10.1002\/acr.23462","volume":"70","author":"Y Zhao","year":"2018","unstructured":"Zhao Y, Wu EY, Oliver MS, Cooper AM, Basiaga ML, Vora SS, et al. Consensus Treatment Plans for Chronic Nonbacterial Osteomyelitis Refractory to Nonsteroidal Antiinflammatory Drugs and\/or With Active Spinal Lesions. Arthritis Care Res (Hoboken). 2018;70(8):1228\u201337.","journal-title":"Arthritis Care Res (Hoboken)"},{"issue":"7","key":"740_CR10","doi-asserted-by":"publisher","first-page":"1063","DOI":"10.1002\/acr.22259","volume":"66","author":"S Ringold","year":"2014","unstructured":"Ringold S, Weiss PF, Colbert RA, DeWitt EM, Lee T, Onel K, et al. Childhood Arthritis and Rheumatology Research Alliance consensus treatment plans for new-onset polyarticular juvenile idiopathic arthritis. Arthritis Care Res (Hoboken). 2014;66(7):1063\u201372.","journal-title":"Arthritis Care Res (Hoboken)"},{"issue":"6","key":"740_CR11","doi-asserted-by":"publisher","first-page":"883","DOI":"10.1007\/s00296-017-3688-3","volume":"37","author":"K Manthiram","year":"2017","unstructured":"Manthiram K, Li SC, Hausmann JS, Amarilyo G, Barron K, Kim H, et al. Physicians\u2019 perspectives on the diagnosis and management of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Rheumatol Int. 2017;37(6):883\u20139.","journal-title":"Rheumatol Int"},{"issue":"3","key":"740_CR12","doi-asserted-by":"publisher","first-page":"251","DOI":"10.1016\/j.clim.2012.09.013","volume":"145","author":"L Shen","year":"2012","unstructured":"Shen L, Suresh L, Lindemann M, Xuan J, Kowal P, Malyavantham K, et al. Novel autoantibodies in Sjogren\u2019s syndrome. Clin Immunol. 2012;145(3):251\u20135.","journal-title":"Clin Immunol"},{"issue":"4","key":"740_CR13","doi-asserted-by":"publisher","first-page":"583","DOI":"10.1002\/acr.24017","volume":"72","author":"JF van Nimwegen","year":"2020","unstructured":"van Nimwegen JF, Mossel E, Delli K, van Ginkel MS, Stel AJ, Kroese FGM, et al. Incorporation of Salivary Gland Ultrasonography Into the American College of Rheumatology\/European League Against Rheumatism Criteria for Primary Sjogren\u2019s Syndrome. Arthritis Care Res (Hoboken). 2020;72(4):583\u201390.","journal-title":"Arthritis Care Res (Hoboken)"},{"issue":"1","key":"740_CR14","doi-asserted-by":"publisher","first-page":"78","DOI":"10.1002\/acr.23839","volume":"72","author":"DS Hammenfors","year":"2020","unstructured":"Hammenfors DS, Valim V, Bica B, Pasoto SG, Lilleby V, Nieto-Gonzalez JC, et al. Juvenile Sjogren\u2019s Syndrome: Clinical Characteristics With Focus on Salivary Gland Ultrasonography. Arthritis Care Res (Hoboken). 2020;72(1):78\u201387.","journal-title":"Arthritis Care Res (Hoboken)"},{"issue":"4","key":"740_CR15","first-page":"578","volume":"32","author":"JC Nieto-Gonzalez","year":"2014","unstructured":"Nieto-Gonzalez JC, Monteagudo I, Bello N, Martinez-Estupinan L, Naredo E, Carreno L. Salivary gland ultrasound in children: a useful tool in the diagnosis of juvenile Sjogren\u2019s syndrome. Clin Exp Rheumatol. 2014;32(4):578\u201380.","journal-title":"Clin Exp Rheumatol"},{"issue":"3","key":"740_CR16","doi-asserted-by":"publisher","first-page":"892","DOI":"10.1093\/rheumatology\/keab579","volume":"61","author":"G Doolan","year":"2022","unstructured":"Doolan G, Faizal NM, Foley C, Al-Obaidi M, Jury EC, Price E, et al. Treatment strategies for Sjogren\u2019s syndrome with childhood onset: a systematic review of the literature. Rheumatology (Oxford). 2022;61(3):892\u2013912.","journal-title":"Rheumatology (Oxford)"},{"issue":"1","key":"740_CR17","doi-asserted-by":"publisher","first-page":"48","DOI":"10.1186\/s12969-019-0342-5","volume":"17","author":"S Shenoi","year":"2019","unstructured":"Shenoi S, Nanda K, Schulert GS, Bohnsack JF, Cooper AM, Edghill B, et al. Physician practices for withdrawal of medications in inactive systemic juvenile arthritis, Childhood Arthritis and Rheumatology Research Alliance (CARRA) survey. Pediatr Rheumatol Online J. 2019;17(1):48.","journal-title":"Pediatr Rheumatol Online J"},{"issue":"1","key":"740_CR18","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1186\/s12969-021-00565-7","volume":"19","author":"R Pooni","year":"2021","unstructured":"Pooni R, Ronis T, Lee T, Investigators C. Telemedicine use by pediatric rheumatologists during the COVID-19 pandemic. Pediatr Rheumatol Online J. 2021;19(1):93.","journal-title":"Pediatr Rheumatol Online J"}],"container-title":["Pediatric Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12969-022-00740-4.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12969-022-00740-4\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12969-022-00740-4.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,9,5]],"date-time":"2022-09-05T14:12:19Z","timestamp":1662387139000},"score":1,"resource":{"primary":{"URL":"https:\/\/ped-rheum.biomedcentral.com\/articles\/10.1186\/s12969-022-00740-4"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,9,5]]},"references-count":18,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2022,12]]}},"alternative-id":["740"],"URL":"https:\/\/doi.org\/10.1186\/s12969-022-00740-4","relation":{},"ISSN":["1546-0096"],"issn-type":[{"value":"1546-0096","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,9,5]]},"assertion":[{"value":"19 May 2022","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"29 August 2022","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"5 September 2022","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The project was declared exempt by the Duke Health Institutional Review Board (Pro00103458).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"RLR\u2019s spouse has financial relationships with Merck and Biogen. LES has received research funds from Bristol Myers Squibb and CARRA. LES serves on the data and safety monitoring board for Sanofi (sarilumab) and UCB (certolizumab). LES is a former President and currently sits on the Registry and Research Oversight Committee for CARRA.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"79"}}