20.500.12556/DiRROS-24635 The incidence of IgG4-related disease in Slovenia single-centre experience Background: Data on the incidence of IgG4-related disease (IgG4-RD) are scarce. Our aim was to determine the incidence of IgG4-RD in a well-defined region. Methods: This retrospective study covered the Ljubljana region over the period from January 2012 to December 2024. A review of cases diagnosed with IgG4-RD was performed at several departments of the University Medical Centre Ljubljana—an integrated secondary/tertiary university teaching hospital (rheumatology, nephrology, angiology, gastroenterology, abdominal surgery, ENT surgery, ophthalmology). While IgG4-RD cases at the Department of Rheumatology were collected prospectively, potential cases at other departments were retrieved by searching electronic medical database for the keyword “IgG4”. In addition, the Institute of Pathology, Faculty of Medicine, University of Ljubljana, provided a list of patients with histological features consistent with IgG4-RD. Year-specific incidence rates and an average incidence rate over the 13-year period were determined. Clinical features of patients were analysed. Results: During the observation period, 58 cases of IgG4-RD were diagnosed. Of these, 35 patients were residents of the Ljubljana region, which had an average adult population of 541,600. The estimated average annual incidence rate of IgG4-RD was 5.0 per million (95% confidence interval: 3.5; 6.9), with year-specific incidence rates fluctuating between 1.8 and 9.3 per million adults. The cases were stratified into four phenotypic categories: pancreato-hepato-biliary (17%), retroperitoneal fibrosis-aortitis (43%), head and neck-limited (14%), and Mikulicz syndrome with systemic involvement (26%). Conclusions: The average annual incidence rate of IgG4-RD was 5 per million adults, with the retroperitoneal fibrosis-aortitis phenotype predominating in our cohort. IgG4-related disease epidemiology demographic incidence rate true false true Angleški jezik Ni določen Neznano 2025-12-09 13:49:42 2025-12-09 13:49:42 2025-12-10 03:45:49 0000-00-00 00:00:00 2025 0 0 Nasl. z nasl. zaslona; Opis vira z dne 13. 10. 2025; str. 1-8 iss. 9, [article no.] 2281 Vol. 13 2025 0000-00-00 Zaloznikova Objavljeno apc 0000-00-00 0000-00-00 0000-00-00 616-002 2227-9059 10.3390/biomedicines13092281 252958723 RAZ_Hocevar_Alojzija_2025.pdf RAZ_Hocevar_Alojzija_2025.pdf 1 FBB7A141F6116AFC90FB9126814A3247 4c7a1aa0bc1002cff1cdfb17dd69d566ec1f305a11ee385ea4951db3fb8e13e4 a4f639fe-d4fd-11f0-94a7-001a4af901a5 https://dirros.openscience.si/Dokument.php?lang=slv&id=36759 https://www.mdpi.com/2227-9059/13/9/2281 0 b6f73f7d-d4fd-11f0-94a7-001a4af901a5 https://dirros.openscience.si/Dokument.php?lang=slv&id=36760 Univerzitetni klinični center Ljubljana 0 0 0