{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T16:57:11Z","timestamp":1773421031997,"version":"3.50.1"},"reference-count":45,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T00:00:00Z","timestamp":1773360000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background\/Objectives: Childhood-onset Sj\u00f6gren disease (cSjD) is a rare autoimmune disorder with heterogeneous manifestations and ongoing diagnostic challenges, as there are no validated paediatric criteria. Our study aims to characterise the clinical, laboratory, and imaging features of children diagnosed with cSjD at a single Romanian paediatric rheumatology centre between 2015 and 2025 and contextualise these findings within the most recent literature. Methods: A retrospective review of 15 consecutive cSjD patients was conducted, including clinical features, autoantibodies, imaging, biopsy findings, treatment, and outcomes. Results: Our cohort showed a significant female predominance (80%) and a broad age range at disease onset (3\u201315 years). Extraglandular manifestations were more common at presentation than glandular phenotypes (53.3% vs. 40%). Lupus-like extraglandular presentations frequently led to initial misdiagnosis as childhood-onset systemic lupus erythematosus (SLE) in our cohort. Sicca symptoms were present at diagnosis in only 3 of 15 patients (20%) and developed later during follow-up in an additional 4 patients (26.7%). Notably, the cohort included novel findings, such as an unprecedented presentation with acute exudative pericarditis complicated by cardiac tamponade. Anti-SSA antibodies and salivary gland ultrasound abnormalities were highly prevalent (86.7% and 100%, respectively). Anti-SSB antibodies were detected in seven patients (46.7%), with titres showing more variability than those of anti-SSA, ranging from just above the positivity threshold to mildly elevated levels. The association with macro-creatine kinase type I was another distinctive feature of this series. Chronic musculoskeletal pain and dryness were our patients\u2019 most frequently reported symptoms at the last assessment, affecting up to 5\/15 (33.3%) in each domain. One patient showed irreversible ocular damage during our study. Conclusions: Extraglandular presentations of cSjD are highly heterogeneous and diagnostically challenging, often occurring without glandular symptoms. Lupus-like systemic features\u2014including facial vasculitic purpura, with or without arthralgia, and occasional pericarditis, as observed in our cohort\u2014may contribute to frequent initial diagnostic misattribution to SLE. Early salivary gland ultrasonography, targeted autoantibody testing, and selective biopsy are essential for timely diagnosis, underscoring the urgent need for paediatric-specific validated classification criteria.<\/jats:p>","DOI":"10.3390\/jcm15062199","type":"journal-article","created":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T15:06:22Z","timestamp":1773414382000},"page":"2199","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Pediatric Sj\u00f6gren Disease: Clinical Features, Diagnostic Challenges, and Outcomes in a Single-Centre Romanian Case Series"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8620-9160","authenticated-orcid":false,"given":"Mihaela","family":"Sparchez","sequence":"first","affiliation":[{"name":"Second Pediatric Discipline, \u201cIuliu Hatieganu\u201d University of Medicine and Pharmacy, 400177 Cluj-Napoca, Romania"},{"name":"Emergency Clinical Hospital for Children Cluj-Napoca, 400370 Cluj-Napoca, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ioana","family":"Filimon","sequence":"additional","affiliation":[{"name":"Emergency Clinical Hospital for Children Cluj-Napoca, 400370 Cluj-Napoca, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mirela","family":"Crisan","sequence":"additional","affiliation":[{"name":"Emergency Clinical Hospital for Children Cluj-Napoca, 400370 Cluj-Napoca, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lidia","family":"Man","sequence":"additional","affiliation":[{"name":"Department of Pediatrics I, \u201cGeorge Emil Palade\u201d University of Medicine, Pharmacy, Sciences and Technology of T\u00e2rgu-Mure\u0219, 540142 T\u00e2rgu Mure\u0219, Romania"},{"name":"Emergency Clinical County Hospital T\u00e2rgu-Mure\u0219, 540136 T\u00e2rgu Mure\u0219, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simona Corina","family":"Senila","sequence":"additional","affiliation":[{"name":"Department of Dermatology, County Emergency Clinical Hospital, \u201cIuliu Ha\u021bieganu\u201d University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ionut","family":"Iarca","sequence":"additional","affiliation":[{"name":"Emergency Clinical Hospital for Children, 500063 Bra\u0219ov, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2240-2540","authenticated-orcid":false,"given":"Laura","family":"Banias","sequence":"additional","affiliation":[{"name":"Pathology Department, George Emil Palade University of Medicine, Pharmacy, Science, and Technology of T\u00e2rgu-Mure\u0219, 540142 T\u00e2rgu Mure\u0219, Romania"},{"name":"Pathology Department, Emergency Clinical County Hospital Targu-Mure\u0219, 540136 T\u00e2rgu Mure\u0219, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreea Liana","family":"Bot (Rachisan)","sequence":"additional","affiliation":[{"name":"Emergency Clinical Hospital for Children Cluj-Napoca, 400370 Cluj-Napoca, Romania"},{"name":"Department 2\u2014Medical Sciences, Faculty of Nursing and Health Sciences, University of Medicine and Pharmacy Iuliu Hatieganu, 400177 Cluj-Napoca, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,3,13]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"4558","DOI":"10.1093\/rheumatology\/keab032","article-title":"Childhood-Onset of Primary Sj\u00f6gren\u2032s Syndrome: Phenotypic Characterization at Diagnosis of 158 Children","volume":"60","author":"Vissink","year":"2021","journal-title":"Rheumatology"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1141","DOI":"10.3899\/jrheum.2024-1048","article-title":"Results of a Nationwide Multicenter Study in Childhood Sj\u00f6gren Disease","volume":"52","author":"Ayduran","year":"2025","journal-title":"J. Rheumatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"3144","DOI":"10.1093\/rheumatology\/keaa757","article-title":"Childhood Sj\u00f6gren Syndrome: Features of an International Cohort and Application of the 2016 ACR\/EULAR Classification Criteria","volume":"60","author":"Basiaga","year":"2021","journal-title":"Rheumatology"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"903","DOI":"10.1016\/j.semarthrit.2020.11.004","article-title":"Childhood Sjogren\u2032s Syndrome: An Italian Case Series and a Literature Review-Based Cohort","volume":"51","author":"Marino","year":"2021","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"85","DOI":"10.55563\/clinexprheumatol\/vxe6h0","article-title":"Differences in Presentation Between Paediatric- and Adult-Onset Primary Sj\u00f6gren\u2032s Syndrome Patients","volume":"39","author":"Legger","year":"2021","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1002\/acr.23839","article-title":"Juvenile Sj\u00f6gren\u2032s Syndrome: Clinical Characteristics with Focus on Salivary Gland Ultrasonography","volume":"72","author":"Hammenfors","year":"2020","journal-title":"Arthritis Care Res."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"2225","DOI":"10.1007\/s10067-017-3745-z","article-title":"A Systematic Review of Primary Sj\u00f6gren\u2032s Syndrome in Male and Paediatric Populations","volume":"36","author":"Virdee","year":"2017","journal-title":"Clin. Rheumatol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"e204","DOI":"10.1016\/S2665-9913(25)00283-8","article-title":"Clinical phenotypes, classification, and long-term outcomes of childhood-onset Sj\u00f6gren\u2032s disease into adulthood: A single-centre cohort study","volume":"8","author":"Ciurtin","year":"2025","journal-title":"Lancet. Rheumatol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1007\/s00431-003-1277-9","article-title":"Primary Sj\u00f6gren syndrome in the paediatric age: A multicentre survey","volume":"162","author":"Cimaz","year":"2003","journal-title":"Eur. J. Pediatr."},{"key":"ref_10","first-page":"689","article-title":"Juvenile onset of primary Sj\u00f6gren\u2032s syndrome: Report of 10 cases","volume":"14","author":"Ostuni","year":"1996","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1136\/annrheumdis-2016-210571","article-title":"2016 American College of Rheumatology\/European League Against Rheumatism classification criteria for primary Sjogren\u2032s syndrome: A consensus and data-driven methodology involving three international patient cohorts","volume":"76","author":"Shiboski","year":"2017","journal-title":"Ann. Rheum. Dis."},{"key":"ref_12","first-page":"343","article-title":"Features of childhood Sjogren\u2032s syndrome in comparison to adult Sjogren\u2032s syndrome: Considerations in establishing child-specific diagnostic criteria","volume":"34","author":"Yokogawa","year":"2016","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_13","first-page":"81","article-title":"Primary Sj\u00f6gren\u2032s syndrome in children and adolescents: Proposal for diagnostic criteria","volume":"17","author":"Tesar","year":"1999","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_14","first-page":"2225","article-title":"Primary Sj\u00f6gren\u2032s syndrome in children and adolescents: Are proposed diagnostic criteria applicable?","volume":"32","author":"Houghton","year":"2005","journal-title":"J. Rheumatol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"3039","DOI":"10.1093\/rheumatology\/keae640","article-title":"Diagnosing a child presenting with symptoms suggesting Sj\u00f6gren\u2032s disease: A tool for clinical practice","volume":"64","author":"Stern","year":"2025","journal-title":"Rheumatology"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1093\/rheumatology\/keae152","article-title":"British Society for Rheumatology guideline on management of adult and juvenile onset Sj\u00f6gren disease","volume":"64","author":"Price","year":"2025","journal-title":"Rheumatology"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"e000022","DOI":"10.1136\/rmdopen-2014-000022","article-title":"EULAR Sj\u00f6gren\u2032s syndrome disease activity index (ESSDAI): A user guide","volume":"1","author":"Seror","year":"2015","journal-title":"RMD Open"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"968","DOI":"10.1136\/ard.2010.143743","article-title":"EULAR Sjogren\u2032s Syndrome Patient Reported Index (ESSPRI): Development of a consensus patient index for primary Sjogren\u2032s syndrome","volume":"70","author":"Seror","year":"2011","journal-title":"Ann. Rheum. Dis."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1103","DOI":"10.1136\/ard.2009.110619","article-title":"EULAR Sj\u00f6gren\u2032s Task Force. EULAR Sjogren\u2032s syndrome disease activity index: Development of a consensus systemic disease activity index for primary Sjogren\u2032s syndrome","volume":"69","author":"Seror","year":"2010","journal-title":"Ann. Rheum. Dis."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"2223","DOI":"10.1002\/art.22658","article-title":"Sj\u00f6gren\u2032s Syndrome Disease Damage Index and disease activity index: Scoring systems for the assessment of disease damage and disease activity in Sj\u00f6gren\u2032s syndrome, derived from an analysis of a cohort of Italian patients","volume":"56","author":"Vitali","year":"2007","journal-title":"Arthritis Rheum."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"967","DOI":"10.1136\/annrheumdis-2019-215024","article-title":"Video clip assessment of a salivary gland ultrasound scoring system in Sjogren\u2032s syndrome using consensual definitions: An OMERACT ultrasound working group reliability exercise","volume":"78","author":"Nicolas","year":"2019","journal-title":"Ann. Rheum. Dis."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1002\/acr.22968","article-title":"Treatment Guidelines for Rheumatologic Manifestations of Sj\u00f6gren\u2032s Syndrome: Use of Biologic Agents, Management of Fatigue, and Inflammatory Musculoskeletal Pain","volume":"69","author":"Carsons","year":"2017","journal-title":"Arthritis Care Res."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1016\/j.rdc.2016.03.010","article-title":"New Treatment Guidelines for Sj\u00f6gren\u2032s Disease","volume":"42","author":"Vivino","year":"2016","journal-title":"Rheum. Dis. Clin. N. Am."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1038\/nrrheum.2016.100","article-title":"Treatment of primary Sj\u00f6gren syndrome","volume":"12","author":"Saraux","year":"2016","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1080\/14397595.2020.1816319","article-title":"Clinical practice guidance for Sj\u00f6gren\u2032s syndrome in pediatric patients (2018)\u2014Summarised and Updated","volume":"31","author":"Tomiita","year":"2021","journal-title":"Mod. Rheumatol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1080\/14397595.2018.1438093","article-title":"Clinical practice guideline for Sjogren\u2032s syndrome 2017","volume":"28","author":"Sumida","year":"2018","journal-title":"Mod. Rheumatol. Jpn. Rheum. Assoc."},{"key":"ref_27","doi-asserted-by":"crossref","unstructured":"Liu, L., Tang, L., Zhang, L., Li, X., Huang, P., Xiong, J., Xiao, Y., and Liu, L. (2022). The first case report of preschool-onset SS\/SLE coexisting with NMOSD of Chinese origin. Front. Immunol., 13.","DOI":"10.3389\/fimmu.2022.887041"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1016\/j.pcl.2018.04.001","article-title":"Systemic Lupus Erythematosus, Sj\u00f6gren Syndrome, and Mixed Connective Tissue Disease in Children and Adolescents","volume":"65","author":"Tarvin","year":"2018","journal-title":"Pediatr. Clin. N. Am."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"R115","DOI":"10.1186\/ar2322","article-title":"Mycophenolate sodium treatment in patients with primary Sjogren syndrome: A pilot trial","volume":"9","author":"Willeke","year":"2007","journal-title":"Arthritis Res. Ther."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1016\/j.ijporl.2018.05.007","article-title":"Oral lesions as presenting feature of childhood Sj\u00f6gren syndrome","volume":"113","author":"Lieberman","year":"2018","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1016\/j.ijporl.2017.07.019","article-title":"Pediatric primary Sj\u00f6gren syndrome presenting with bilateral ranulas: A case report and systematic review of the literature","volume":"101","author":"Means","year":"2017","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_32","doi-asserted-by":"crossref","unstructured":"Takagi, Y., Katayama, I., Eida, S., Sasaki, M., Shimizu, T., Sato, S., Hashimoto, K., Mori, H., Otsuru, M., and Umeda, M. (2023). Three Signs to Help Detect Sj\u00f6gren\u2032s Syndrome: Incidental Findings on Magnetic Resonance Imaging and Computed Tomography. J. Clin. Med., 12.","DOI":"10.3390\/jcm12206487"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"E2020042127","DOI":"10.1542\/peds.2020-042127","article-title":"Childhood-Onset Sj\u00f6gren Syndrome Presenting as Pulmonary Hemorrhage","volume":"148","author":"Wang","year":"2021","journal-title":"Pediatrics"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"S43","DOI":"10.1007\/s10067-009-1121-3","article-title":"Hemolytic uremic syndrome and pericarditis as early manifestations of primary Sj\u00f6gren\u2032s syndrome","volume":"28","author":"Chen","year":"2009","journal-title":"Clin. Rheumatol."},{"key":"ref_35","doi-asserted-by":"crossref","unstructured":"Duarte, F., Oliveira, L., Fontes, T., Ramos, S., Dourado, R., and Martins, D. (2023). Chronic constrictive pericarditis: A rare cardiac involvement in primary Sj\u00f6gren\u2032s syndrome. BMC Cardiovasc. Disord., 23.","DOI":"10.1186\/s12872-023-03491-6"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1136\/ard.55.7.450","article-title":"Cardiac manifestations in primary Sj\u00f6gren\u2032s syndrome","volume":"55","author":"Pokorny","year":"1996","journal-title":"Ann. Rheum. Dis."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1258\/acb.2012.011270","article-title":"Detection of creatine kinase macroenzymes","volume":"49","author":"Davidson","year":"2012","journal-title":"Ann. Clin. Biochem."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1111\/imj.12710","article-title":"Macro-creatine kinase: A neglected cause of elevated creatine kinase","volume":"45","author":"Aljuani","year":"2015","journal-title":"Intern. Med. J."},{"key":"ref_39","doi-asserted-by":"crossref","unstructured":"Thatayatikom, A., Jun, I., Bhattacharyya, I., Berg, K., Lee, Y.J., Kim, Y., Adewumi, A., Zhang, W., Thatayatikom, S., and Shah, A. (2021). The Diagnostic Performance of Early Sjogren\u2032s Syndrome Autoantibodies in Juvenile Sjogren\u2032s Syndrome: The University of Florida Pediatric Cohort Study. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.704193"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1169","DOI":"10.1136\/ard-2023-224936","article-title":"Novel autoantibodies help diagnose anti-SSA antibody negative Sj\u00f6gren disease and predict abnormal labial salivary gland pathology","volume":"83","author":"Parker","year":"2024","journal-title":"Ann. Rheum. Dis."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"102087","DOI":"10.1016\/j.berh.2025.102087","article-title":"Advances in imaging techniques for Sjogren\u2032s disease","volume":"39","year":"2025","journal-title":"Best Pract. Res. Clin. Rheumatol."},{"key":"ref_42","first-page":"2283","article-title":"Ultrasonographic evaluation of lacrimal glands in patients with primary Sj\u00f6gren\u2032s syndrome","volume":"40","author":"Kim","year":"2022","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_43","first-page":"2520","article-title":"Clinical utility of salivary and lacrimal gland ultrasonography in primary Sj\u00f6gren\u2032s syndrome","volume":"42","author":"Kim","year":"2024","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1115","DOI":"10.1080\/02713683.2024.2349637","article-title":"Radiological Imaging of the Lacrimal Gland in Sjogren\u2032s Syndrome: A Systematic Review and Meta-Analysis","volume":"49","author":"Singh","year":"2024","journal-title":"Curr. Eye Res."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"105825","DOI":"10.1016\/j.jbspin.2024.105825","article-title":"Diagnostic value of minor salivary gland biopsy for Sj\u00f6gren\u2032s syndrome in children: A monocentric retrospective study over 10 years","volume":"92","author":"Adeline","year":"2025","journal-title":"Jt. Bone Spine"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/6\/2199\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T15:19:29Z","timestamp":1773415169000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/6\/2199"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,13]]},"references-count":45,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2026,3]]}},"alternative-id":["jcm15062199"],"URL":"https:\/\/doi.org\/10.3390\/jcm15062199","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,13]]}}}