Autoinflammatory & rare systemic diseases
Clinical and histopathological features of a population-based cohort of 95 consecutive patients with immunoglobulin G4-related disease in Stockholm, Sweden, during a five-year period
Authors' conclusion
IgG4-RD in Stockholm County shows a pancreatohepatobiliary-renal dominant pattern, with relatively few glandular cases compared to Asian and US cohorts. Histopathological confirmation was supportive in most biopsied patients, although many affected organs were not biopsied.
Study context
- Population
- Patients with an ICD-10-SE diagnosis of immunoglobulin G4-related disease (IgG4-RD) recorded 2019–2023 in Stockholm County, Sweden, identified from the Swedish National Patient Register112 registered, 95 confirmed
- Participants
- 95 patients
- Outcome
- Epidemiology, organ involvement, histopathology and management of IgG4-RD
Methodological detailspopulation-based, register-based (Swedish National Patient Register), systematic medical record review
- Population-based
- Register-based (Swedish National Patient Register)
- Systematic medical record review
Main finding
As reported in the abstract
Male
68%
Involvement of two or more organs
60%
Elevated serum IgG4
71%
Underwent biopsy
76%
Observational data: associations, not effects.
Further findings
Comorbidities at diagnosis
- Similar to the general older population, with elevated prevalence of nasal polyposis and asthma
Treatment and prognosis
- Most received systemic GC, alone or with RTX
- Prognosis favourable, with high remission rates
Original publication
Clinical and histopathological features of a population-based cohort of 95 consecutive patients with immunoglobulin G4-related disease in Stockholm, Sweden, during a five-year period. Scand J Rheumatol. Published 16 June 2026. doi:10.
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