Benefits of Early Versus Late Initiation of Intravenous Immunoglobulin in the Treatment of Patients With Anti–3‐Hydroxy‐3‐Methylglutaryl‐Coenzyme A Reductase Immune‐Mediated Necrotizing Myopathy
An erratum to this article was published (Arthritis Care Res (Hoboken), Mar 2025 ↗)
Authors' conclusion
Delay in IVIG treatment may lead to the development of permanent residual weakness and long-term disability.
Study context
- Population
- Adults with anti-HMGCR Immune-mediated Necrotizing Myopathy (IMNM) (2016 European Neuromuscular Centre criteria), Oregon Health and Science University Myositis Center, September 2016–October 2022
- Participants
- 31 adults
- Exposure
- Nondelayed vs delayed initiation of IVIG
- Nondelayed treatmentn = 19
- Delayed treatmentn = 12reference
- Outcome
- Response to treatment by limited total improvement score (TIS), 2016 ACR/EULAR myositis response criteria
- Follow-up
- 3, 6 and 12 months
Methodological detailsretrospective chart review, single myositis centre
- Retrospective chart review
- Single myositis centre
Main finding
As reported in the abstract
TIS improvement
- Nondelayed treatment vs Delayed treatment: P = 0.002 at 3 months
- Nondelayed treatment vs Delayed treatment: P = 0.019 at 6 months
- Nondelayed treatment vs Delayed treatment: P = 0.001 at 12 months
Observational data: associations, not effects.
Further findings
Walker or wheelchair use at 0 months
Events
| Outcome | Nondelayed treatment | Delayed treatment |
|---|---|---|
| Permanent residual muscle weakness requiring walker or wheelchair · 12 months | 0 | 7 |
- Serum creatine kinase (CK) at 0-month time point · 0 months
- P = 0.016
- Time between diagnosis and IVIG treatment · significantly longer with delayed treatment
- P < 0.001
- Serum CK at 0-month time point: lower serum CK with delayed treatment; comparable at disease onset (P > 0.999)
Manual muscle testing 8 (MMT8) · 12 months
- Significant improvement in MMT8 with nondelayed treatment
- P < 0.001
Serum CK · 3, 6 and 12 months
- Average serum CK did not significantly differ between groups
Original publication
Benefits of Early Versus Late Initiation of Intravenous Immunoglobulin in the Treatment of Patients With Anti–3‐Hydroxy‐3‐Methylglutaryl‐Coenzyme A Reductase Immune‐Mediated Necrotizing Myopathy. Arthritis Care Res (Hoboken). Published 11 September 2024. doi:10. 1002/ acr. 25406
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