Autoinflammatory & rare systemic diseases
Immunosuppressive agents or intravenous immunoglobulin in addition to glucocorticoids in the treatment of Susac syndrome: a French national cohort study
An erratum to this article was published (Lancet Rheumatol, Dec 2024 ↗)
Main finding · First relapse within 36 months (relapse-free survival)Result — go to the resultHR for first relapse 1.11 95% CI 0.56–2.17Authors' conclusion
The combination of glucocorticoids with immunosuppressive agents or intravenous immunoglobulin did not appear to reduce the risk of Susac syndrome relapse compared with glucocorticoids alone.
Study context
- Population
- Patients aged ≥18 years with Susac syndrome referred to the French reference centre (Bichat-Claude Bernard Hospital, Paris), 2011–202241 women · 23 men
- Participants
- 64 patients
- Exposure
- GC plus immunosuppressive agents or IV immunoglobulin vs GC alone
- Outcome
- First relapse (new clinical symptoms or signs and new abnormalities on retinal angiography, audiometry or brain MRI, necessitating treatment intensification)
- Follow-up
- Within 36 months from first day of treatment
Methodological detailsnational, consecutive patients, single reference centre
- National
- Consecutive patients
- Single reference centre
Main finding
As reported in the abstract
First relapse within 36 months (relapse-free survival)
HR for first relapse
1.11
95% CI 0.56–2.17
P = 0.76
Glucocorticoids in combination with immunosuppressive agents or intravenous immunoglobulinvs Glucocorticoids alone
HR for first relapse 1.11 (95% CI 0.56–2.17); P = 0.76
- Patients with a first relapse (overall)
- 46 (72%) of 64
- Median relapse-free survival time (overall), months
- 3.96 (95% CI 2.24-16.07)
Observational data: associations, not effects.
Further findings
| Outcome | HR for second relapse (95% CI) | P value | |
|---|---|---|---|
| Second relapse in patients who first relapsed on GC alone: second-line immunosuppressive agents or intravenous immunoglobulinvs not | 2.66 (0.63–11.18) | P = 0.18 | |
| 0.050.21520HR for second relapse (log scale) |
Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.
Original publication
Immunosuppressive agents or intravenous immunoglobulin in addition to glucocorticoids in the treatment of Susac syndrome: a French national cohort study. Lancet Rheumatol. Published 18 September 2024. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →