ArticlePediatric rheumatology online journal2023
Childhood-onset primary Sjögren's syndrome in a tertiary center in China: clinical features and outcome.
Article in Pediatric rheumatology online journal, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
15 citing papers in PubMed, 1 synthesis or guideline pooled it, 24 citations in OpenAlex.
- British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease.Rheumatology (Oxford, England) · 2025Guideline
- Managing Sjögren's Disease in Pediatric Patients.Paediatric drugs · 2026Review
- Minor salivary gland biopsies in childhood-onset Sjögren's disease show greater inflammation and more frequent fibrotic changes than in adult-onset Sjögren's disease.Arthritis research & therapy · 2026Article
- Pediatric Sjögren's Disease: Literature Review and Diagnostic Challenges in an Uncommon Case.Diagnostics (Basel, Switzerland) · 2026Article
- Juvenile Sjögren disease: time for age-specific classification criteria.Pediatric rheumatology online journal · 2026Article
- Nephrogenic Diabetes Insipidus Presenting with Hypokalemic Paralysis in an Adolescent with Primary Sjögren's Syndrome: A Case Report.Kidney & blood pressure research · 2026Article
- Childhood Sjögren's Disease: A Literature Review of an Underrecognized Autoimmune Entity in Pediatric Rheumatology.Cureus · 2025Review
- Childhood-onset primary Sjögren's Syndrome presenting as nephrotic syndrome: a case report and literature review.BMC pediatrics · 2025Review
- Multidimensional assessment of Juvenile Sjögren disease.Clinical rheumatology · 2025Article
- A Chinese girl with neuromyelitis optica spectrum disorder coexisting with primary Sjogren's syndrome: a case report and literature review.Frontiers in immunology · 2025Review
- Imaging the Micron: New Directions in Diagnosis with Ultra-High-Frequency Ultrasound.Diagnostics (Basel, Switzerland) · 2024Article
- Kidney manifestations of pediatric Sjögren's syndrome.Pediatric nephrology (Berlin, Germany) · 2024Article
- An exceptional cause of polyuria-polydipsia syndrome in a 10-year-old boy.Pediatric nephrology (Berlin, Germany) · 2024Article
- Microvascular status in juvenile Sjögren's disease: the first nailfold videocapillaroscopy investigation.Clinical rheumatology · 2024Article
- A single-center study of clinical features of pediatric Sjögren's syndrome.Pediatric rheumatology online journal · 2023Article
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Authors and funding
13 authors at 1 institution in 1 country.
Funding
Abstract
objectivesTo characterize the clinical features and outcomes of childhood-onset primary Sjögren's syndrome (pSS).
methodsPatients less than 18 years old who were diagnosed with pSS by paediatric rheumatologists were included, and all patients were applied the 2002 American-European Consensus Group (ACEG) criteria, the 2016 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for pSS, or the 1999 proposed juvenile pSS criteria. The electronic medical records of patients with pSS from 2013 to 2020 were collected and analysed.
resultsThirty-nine patients were included. Of them, 27 (69.2%), 38 (97.4%) and 35 (89.7%) patients fulfilled the AECG criteria, ACR/EULAR criteria and proposed juvenile pSS criteria, respectively. The female:male ratio was 3.9:1. The median ages at first signs or symptoms and at diagnosis were 9.2 (4.7, 14.5) years and 10.9 (6.3, 15.0) years, respectively. The main clinical manifestations were rash or purpura (20, 51.3%), followed by fever (12, 30.8%), glandular enlargement/recurrent parotitis (10, 25.6%), and dry mouth and/or dry eyes (9, 23.1%). Twenty-eight (56.4%) patients had systemic damage, the most common of which was haematological involvement (14, 35.9%), followed by hepatic (13, 33.3%) and renal involvement (8, 20.5%). Thirty-eight (97.4%) patients underwent labial minor salivary gland biopsy, and all exhibited focal lymphocytic sialadenitis. All patients had a global ESSDAI score ≥ 1 at diagnosis, and the median total score at diagnosis was 8 (2, 31). Thirty-six (92.3%) patients were followed up for a median time of 23.6 (7.9, 79.5) months, and three patients developed systemic lupus erythematosus (SLE) at follow-up times of 13.3, 38.8 and 63.8 months.
conclusionsThe presentation of childhood-onset pSS is atypical, and extraglandular manifestations and systemic involvement are more common than in adult-onset pSS. Labial salivary gland biopsy is vital for patients with probable pSS. Some patients may develop SLE over time.
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