Autoinflammatory & rare systemic diseases
IgG4-related uveitis. A French cohort and literature review
Authors' conclusion
Uveitis may be one of the initial symptoms of IgG4-RD, and IgG4-RD should be considered in the diagnostic workup of uveitis.
Study context
- Population
- Patients with uveitis and a concomitant definite diagnosis of igG4-related disease (IgG4-RD), from the French national IgG4-RD and systemic fibrosis database and from the literature
- Participants
- 16 patients30 uveitis episodes
- Outcome
- Pattern of IgG4-related uveitis
Methodological detailsnationwide, PubMed literature review
- Nationwide
- PubMed literature review
Main finding
As reported in the abstract
- Uveitis cases among total IgG4-RD patients in the national database
- 3 %
- Uveitis inaugural in IgG4-RD (median 9 months before other symptoms)
- 4/16 (25 %)
- Uveitis concurrent with other IgG4-related symptoms (median 15 months before diagnosis)
- 9/16 (56 %)
- Uveitis appearing during follow-up (median 57 months after first symptoms)
- 3/16 (19 %)
- Uveitis during follow-up associated with IgG4-RD in other organs
- 6/9 (67 %)
- Bilateral
- 8/16 (50 %)
- Granulomatous
- 5/10 (50 %)
- Anterior
- 8/13 (62 %)
- Intermediate
- 3/13 (23 %)
- Panuveitis
- 4/13 (31 %)
- Median serum IgG4 at diagnosis
- 3.2 g/L
Observational data: associations, not effects.
Further findings
At least one uveitis relapse during follow-up (median 6 years)
- Patients with ≥1 relapse
- 8/16 (50 %)
Treatment and remission (29/30 flares with treatment data)
- Steroids used
- 28/29 (97 %)
- Remission with steroids
- 16/28 (57 %)
- MTX (second/third line, with steroids) used
- 6/29 (21 %)
- Remission with MTX
- 4/6 (67 %)
- RTX (second/third line, with steroids) used
- 5/29 (17 %)
- Remission with RTX
- 4/5 (80 %)
- Uveitides requiring ≥2 lines of treatment for remission induction
- One third
Original publication
IgG4-related uveitis. A French cohort and literature review. Semin Arthritis Rheum. Published 11 November 2023. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →