Fellows’ Forum Case Report: Necrotizing Autoimmune Myopathy

The second patient initially presented with severe respiratory muscle weakness leading to hypoxic hypercapnic respiratory failure and cardiac involvement. Therefore, we gave him a combination of IVIG and high-dose steroids, and he responded well.

Conclusion

HMGCR Ab-related IMNM can present with severe respiratory muscle weakness that leads to hypoxic hypercapnic respiratory failure, dysphagia and cardiac involvement, which can contribute to significant morbidity and mortality. Persistently high CPK levels, progressive muscle weakness, dysphagia and respiratory muscle involvement include some of the indicators of severe disease. Doctors must promptly identify such patients and manage them with aggressive immunosuppression.

Shivani Garg, MD, is an assistant professor in the Department of Internal Medicine, Division of Rheumatology at the School of Medicine and Public Health in the University of Wisconsin in Madison, Wis.

Suzana Alex John, MD, is a fellow in the Department of Rheumatology at Emory University in Atlanta.

Frehiywot Ayele, MD, is an assistant professor in the Department of Rheumatology at Emory University in Atlanta.

References

1. Carroll MB, Newkirk MR, Sumner NS. Necrotizing autoimmune myopathy: A unique subset of idiopathic inflammatory myopathy. J Clin Rheumatol. 2016 Oct;22(7):376–380.
2. Mohassel P, Mammen AL. Statin-associated autoimmune myopathy and anti-HMGCR autoantibodies. Muscle Nerve. 2013 Oct;48(4):477–483.
3. Basharat P, Christopher-Stine L. Immune-mediated necrotizing myopathy: Update on diagnosis and management. Curr Rheumatol Rep. 2015 Dec;17(12):72.
4. Albayda J, Christopher-Stine L. Identifying statin-associated autoimmune necrotizing myopathy. Cleve Clin J Med. 2014 Dec;81(12):736–741.
5. Hoogendijk JE1, Amato AA, Lecky BR, et al. 119th ENMC international workshop: Trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis. Neuromuscul Disord. 2004 May;14(5):337–345.
6. Ramanathan S, Langguth D, Hadry T, et al. Clinical course and treatment of anti-HMGCR antibody-associated necrotizing autoimmune myopathy. Neurol Neuroimmunol Neuroinflamm. 2015 Apr 2;2(3):e96.
7. Mammen AL. Statin-associated autoimmune myopathy. N Engl J Med. 2016 Feb 18;374(7):664–669.
8. Meriggioli MN. The clinical spectrum of necrotizing autoimmune myopathy: A mixed bag with blurred lines. JAMA Neurol. 2015 Sep;72(9), 977–979.
9. Giudizi MG, Cammelli D, Vivarelli E, et al. Anti-HMGCR antibody-associated necrotizing myopathy: Diagnosis and treatment illustrated using a case report. Scand J Rheumatol. 2016 Oct;45(5):427–429.
10. Christopher-Stine L, Casciola-Rosen LA, Hong G, et al. A novel autoantibody recognizing 200-kd and 100-kd proteins is associated with an immune-mediated necrotizing myopathy. Arthritis Rheum. 2010 Sep;62(9):2757–2766.
11. Mammen AL, Chung T, Christopher-Stine L, et al. Autoantibodies against 3-hydroxy-3-methylglutaryl-coenzyme A reductase in patients with statin-associated autoimmune myopathy. Arthritis Rheum. 2011 Mar;63(3):713–721.
12. Musset L, Allenbach Y, Benveniste O, et al. Anti-HMGCR antibodies as a biomarker for immune-mediated necrotizing myopathies: A history of statins and experience from a large international multi-center study. Autoimmun Rev. 2016 Oct;15(10):983–993.
13. Lahaye C, Beaufrére AM, Boyer O, et al. Immune-mediated myopathy related to anti 3-hydroxy-3-methylglutaryl-coenzyme A reductase antibodies as an emerging cause of necrotizing myopathy induced by statins. Joint Bone Spine. 2014 Jan;81(1):79–82.
14. Mammen AL. Autoimmune myopathies: Autoantibodies, phenotypes and pathogenesis. Nat Rev Neurol. 2011 Jun 8;7(6):343–354.
15. Werner JL, Christopher-Stine L, Ghazarian SR, et al. Antibody levels correlate with creatine kinase levels and strength in anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase-associated autoimmune myopathy. Arthritis Rheum. 2012 Dec;64(12):4087–4093.
16. Mammen L, Pak K, Williams EK, et al. Rarity of anti-3-hydroxy-3-methylglutarly-coenzyme A reductase antibodies are rare in statin users, including those with self-limited musculoskeletal side-effects. Arthritis Care Res (Hoboken). 2012 Feb;64(2):269–272.
17. Levenson VV. Biomarkers for early detection of breast cancer: What, when and where? Biochim Biophys Acta. 2007 Jun;1770(6):847–856.
18. Misek DE, Kim EH. Protein biomarkers for the early detection of breast cancer. Int J Proteomics. 2011;2011.
19. Allenbach Y, Drouot L, Rigolet A, et al. Anti-HMGCR autoantibodies in European patients with autoimmune necrotizing myopathies: Inconstant exposure to statin. Medicine (Baltimore). 2014 May;93(3):150–157.
20. Kassardjian CD, Lennon VA, Alfugham NB, et al. Clinical features and treatment outcomes of necrotizing autoimmune myopathy. JAMA Neurol. 2015 Sep;72(9):996–1003.