Inflammatory myopathies

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 Retrospective cohort study

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

OutcomeNondelayed treatmentDelayed treatment
Permanent residual muscle weakness requiring walker or wheelchair · 12 months07
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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