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Ayesha SM, Meena AK, Vangala N, et al. Necrotizing Autoimmune Myopathy: Clinicopathologic Study from a Single Tertiary Care Centre. Ann Indian Acad Neurol. 2018;21(1):62-67doi: 10.4103/aian.AIAN_389_17.
Ayesha, S. M., Meena, A. K., Vangala, N., Rajasekhar, L., Kaul, S., Borgahain, R., & Uppin, M. S. (2018). Necrotizing Autoimmune Myopathy: Clinicopathologic Study from a Single Tertiary Care Centre. Annals of Indian Academy of Neurology, 21(1), 62-67. https://doi.org/10.4103/aian.AIAN_389_17
Ayesha, Sobiya Mahnaz, et al. "Necrotizing Autoimmune Myopathy: Clinicopathologic Study from a Single Tertiary Care Centre." Annals of Indian Academy of Neurology vol. 21,1 (2018): 62-67. doi: https://doi.org/10.4103/aian.AIAN_389_17
Ayesha SM, Meena AK, Vangala N, Rajasekhar L, Kaul S, Borgahain R, Uppin MS. Necrotizing Autoimmune Myopathy: Clinicopathologic Study from a Single Tertiary Care Centre. Ann Indian Acad Neurol. 2018 Jan-Mar;21(1):62-67. doi: 10.4103/aian.AIAN_389_17. PMID: 29720800; PMCID: PMC5909148.
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Ann Indian Acad Neurol. 2018 Jan-Mar;21(1):62-67. doi: 10.4103/aian.AIAN_389_17.

Necrotizing Autoimmune Myopathy: Clinicopathologic Study from a Single Tertiary Care Centre.

Annals of Indian Academy of Neurology

Sobiya Mahnaz Ayesha, A K Meena, Navatha Vangala, Liza Rajasekhar, Subhash Kaul, Rupam Borgahain, Megha S Uppin

Affiliations

  1. Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
  2. Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
  3. Department of Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.

PMID: 29720800 PMCID: PMC5909148 DOI: 10.4103/aian.AIAN_389_17

Abstract

BACKGROUND: Idiopathic inflammatory myopathies (IIMs) are a group of chronic, autoimmune disorders which include a new entity, necrotizing autoimmune myopathy (NAM). NAM lacks inflammation and presents with markedly elevated creatinine phosphokinase (CPK) levels. It is associated with connective tissue diseases (CTDs), statin use, malignancies, and most cases are idiopathic.

OBJECTIVES: The objectives of this study are to describe the clinicopathologic features in muscle biopsy-proven cases of NAM. To emphasize the role of laboratory parameters such as CPK levels and myositis profile in the diagnosis of NAM.

MATERIALS AND METHODS: This is a retrospective study including 15 patients of NAM diagnosed on muscle biopsy over a period of 2 years. The slides of the biopsies were reviewed, and clinical data, electromyography findings, and CPK levels were obtained. Myositis profile was done.

RESULTS: Necrotizing myopathy accounted for 13.63% (15 cases) of total inflammatory myopathies (110 cases) in the study. These were grouped into CTD-associated NAM, statin-associated NAM, paraneoplastic NAM and idiopathic NAM which was the common type. All cases presented with progressive proximal muscle weakness and had markedly elevated CPK levels. Anti-3-hydroxy-3-methyl-glutaryl-coenzyme A reductase and antisignal recognition particle antibodies were seen to be positive in six patients. Muscle biopsies showed predominant fiber necrosis with significant fiber degeneration and regeneration in the absence of inflammation. All patients received immunotherapy with significant improvement was seen in six patients with two mortalities.

CONCLUSION: Necrotizing myopathy is a new addition to the spectrum of IIM. Clinicopathologic correlation is important for appropriate diagnosis. It is found to be refractory to corticosteroids monotherapy. The course of illness is not uniform, and in some patients, there can be rapid worsening with mortality.

Keywords: 3-Hydroxy-3-methyl-glutaryl-coenzyme A reductase; muscle biopsy; necrotizing myopathy; signal recognition particle antibodies; statins

Conflict of interest statement

There are no conflicts of interest.

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