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Emerging Investigator Awardee
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Poster Session

Periodic Fever Syndromes, Autoinflammatory Diseases, Still’s Disease and MAS/HLH

Poster Session C

Session: (2015–2051) Miscellaneous Rheumatic & Inflammatory Diseases Poster III

2015: Clinical features of IgG4-related disease - a single centre experience

Tuesday, October 28, 2025
10:30 AM - 12:30 PM Central Time
Location: Hall F1

    Abstract Poster Presenter(s)

  • AH

    Alojzija Hocevar, MD, PhD

    University Medical Center Ljubljana
    Ljubljana, Slovenia

    Disclosure(s): No financial relationships with ineligible companies to disclose


Background/Purpose: IgG4-related diseases (IgG4-RD) is rare and unique fibroinflammatory disorder. The aim of our study was to study the characteristics of IgG4-RD cohort diagnosed and followed at our secondary/tertiary medical centre.

Methods: This retrospective study covered a period from January 2012 to December 2024. A review of medical records of adult patients diagnosed with IgG4-RD was performed and patient’s demographics, comorbidities and IgG4-RD features at presentation were extracted. Data were analysed with descriptive statistical methods.

Results: During a 13-year observation period, 58 patients with IgG4-RD were identified (46 (79.3%) males, median (IQR) age at diagnosis 63.0 (51.3; 68.9) years). Ten patients had a history of diabetes mellitus, 10 of asthma and 5 of atopy. Median (IQR) duration of symptoms attributed to IgG4RD before the diagnosis was 6 (3; 18) months.
Table 1 shows characteristics of our IgG4-RD cohort at diagnosis. The average number of organs and/or regions affected was 2 (ranging from 1 to 7). Thirty-seven (63.8%) patients had predominantly proliferative and 21 (36.2%) predominantly fibrotic phenotypes. We classified 10 (17.2%) patients as pancreato-hepato-biliary disease, 25 (43.1%) as retroperitoneal fibrosis and/or aortitis, 6 (10.3%) as head and neck limited disease and 15 (25.9%) as Mikulicz syndrome with systemic involvement. In 2 patients the disease could not be classified. Forty-seven patients had an organ biopsy, and histopathological features were consistent with IgG4-RD in 36 (76.6%), and suspicious of IgG4-RD in 4 (8.5%) patients. 2019 ACR-EULAR IgG4RD classification criteria were fulfilled in 40 (69.0%) patients.
A total of 44 patients (75.9%) received systemic therapy: 41 patients were treated with systemic glucocorticoid, 25 with an immunomodulatory medication (3 of them as therapy and 22 concomitantly with glucocorticoids). Nine patients received surgical therapy. Five patients were observed only.

Conclusion: Our IgG4-RD cohort was dominated by middle-aged men with retroperitoneal fibrosis and/or aortitis phenotype.