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Purpose: Our study aimed to make a bibliometric analysis on Sjögren's syndrome-related dry eye disease (SS-DED), explore its potential research hotspots, and provide critical information for future research interest and undeveloped topics in this field, which can benefit clinicians and researchers., Methods: We extracted all publications relating to SS-DED from 2003 to 2022 from the Web of Science Core Collection database. Original articles and reviews in English were included. The contributions of different countries, institutions, journals, and authors were compared, and the research hotspots were visualized for network analysis through GraphPad Prism, CiteSpace, and VOSviewer., Results: We enrolled a total of 987 publications. The United States contributed the most publications (281, 28.5%), followed by China (157, 15.9%) and Japan (80, 8.11%). Publications from the United States were cited more frequently (13,060 citations), with the highest H-index of 57. China ranked second in the total number of publications, the papers were not cited frequently (3790 citations), and the H-index ranked second (H-index = 31). PLoS One (32, 3.24%) published the most papers, and the University of California system had the highest number of publications (45, 4.56%). Bootsma H from the Netherlands published the most papers. The trend of research hotspots evolved mainly from the basic manifestation to pathogenesis and treatment of SS-DED and paid more attention to distinguishing SS-DED from that dry eye disease without SS., Conclusions: Based on the bibliometric, co-citation, and network analyses in this study, we obtained the annual publications and citations, the growth trends of publications, the productivity of countries, organizations, journals, and authors, high-quality publications, and the emerging hotspots of SS-DED, which may open new doors for promising research directions., (© 2023. The Author(s), under exclusive licence to Springer Nature B.V.)

Objectives: To characterize the clinical features and outcomes of childhood-onset primary Sjögren's syndrome (pSS). Methods: Patients 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. Results: Thirty-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. Conclusions: The 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. [ABSTRACT FROM AUTHOR]