A rare disease with many faces: a multicentre registry of IgG4-related disease in children
Authors' conclusion
IgG4-RD has a wide variety of clinical manifestations; however, in children, the most common presentation was orbital involvement. The 2020 RCD criteria had a better performance whereas the 2019 ACR/EULAR classification criteria performed poorly in paediatric patients.
Study context
- Population
- Children with IgG4-related disease (IgG4-RD) diagnosed by the 2011 comprehensive diagnostic criteria, 13 paediatric rheumatology centres19 females · 16 males
- Participants
- 35 children
- Exposure
- 2019 ACR/EULAR vs 2020 revised comprehensive diagnostic (RCD)
- Outcome
- Characteristics of paediatric IgG4-RD
Methodological detailsweb-based registry, 13 paediatric rheumatology centres
- Web-based registry
- 13 paediatric rheumatology centres
Main finding
As reported in the abstract
- Median age at diagnosis (25p-75p 9.9-15.2), years
- 13.3
- Eye involvement
- n = 21, 60%
- Lymph node involvement
- n = 12, 34.3%
- Musculoskeletal involvement
- n = 12, 34.3%
- Neurological involvement
- n = 9, 25.7%
- Cluster: eye involvement
- n = 11, 31.4%
- Cluster: eye involvement and neurological findings
- n = 15, 42.9%
- Cluster: pancreato-hepatobiliary disease and lymph node involvement
- n = 9, 25.7%
- High serum IgG4 levels (19 out of 28 patients)
- 67.8%
Observational data: associations, not effects.
Further findings
Sensitivity of classification/diagnostic criteria
Sensitivity
Treatment
- All patients except one received corticosteroids; azathioprine the most preferred steroid-sparing agent
Original publication
A rare disease with many faces: a multicentre registry of IgG4-related disease in children. Rheumatology (Oxford). Published 19 September 2024. doi:10.
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