Key result
Treatment with intravenous immunoglobulin and prednisone resolved cardiomyopathy, improving ejection fraction from 40% to 60% in a patient with anti-HMGCR immune-mediated necrotizing myopathy.
Why the study?
Cardiac involvement is well reported in anti-SRP IMNM, but emerging evidence suggests it may also occur in the anti-HMGCR subgroup.
Does treatment with IVIG and prednisone improve cardiac involvement in a patient with anti-HMGCR immune-mediated necrotizing myopathy?
Case Report (n=1)
Does treatment with IVIG and prednisone improve cardiac involvement in a patient with anti-HMGCR immune-mediated necrotizing myopathy?
This case highlights the potential for reversible cardiac involvement in anti-HMGCR immune-mediated necrotizing myopathy and underscores the need for routine cardiac workup in these patients.
Warrants vigilance for cardiac involvement in anti-HMGCR IMNM; single case leaves open need for confirmatory studies before screening changes.
Immune-mediated necrotizing myopathy (IMNM) is categorized into three groups: anti-3-hydroxy-3-methylglutaryl coenzyme A reductase (HMGCR) IMNM, anti-signal recognition particle (SRP) IMNM, and seronegative IMNM. Cardiac involvement has been reported in a significant segment of patients with IMNM of the anti-SRP type. Emerging evidence now suggests that cardiac involvement is also implicated in the anti-HMGCR subgroup. In this report, we present a case of anti-HMGCR IMNM with cardiac involvement demonstrated by elevated troponin levels, a low ejection fraction of 40%, and regional wall motion abnormalities in the inferior, inferolateral, anteroseptal, inferoseptal, and anterolateral myocardial walls, as visualized on echocardiography. These findings markedly improved after treatment with intravenous immunoglobulin (IVIG) and prednisone. This case and other recent reports highlight the need for a cardiac workup in patients diagnosed with anti-HMGCR IMNM.
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Liu et al. (2022) conducted a case report in Anti-HMGCR immune-mediated necrotizing myopathy (IMNM) with cardiac involvement (n=1). Intravenous immunoglobulin (IVIG) and prednisone was evaluated on Clinical and echocardiographic improvement. Treatment with intravenous immunoglobulin and prednisone resolved cardiomyopathy, improving ejection fraction from 40% to 60% in a patient with anti-HMGCR immune-mediated necrotizing myopathy.
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