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Necrotizing Autoimmune Myopathy: Clinicopathologic Study from a Single Tertiary Care Centre
Sobiya Mahnaz Ayesha1, A K Meena2, Navatha Vangala1
1Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
Annals of Indian Academy of Neurology
|May 4, 2018
Summary
Necrotizing autoimmune myopathy (NAM) is a distinct autoimmune disorder characterized by muscle fiber necrosis without inflammation, often presenting with high creatine phosphokinase (CPK) levels. Clinicopathologic correlation is crucial for diagnosis and management.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Idiopathic inflammatory myopathies (IIMs) encompass necrotizing autoimmune myopathy (NAM), a condition lacking inflammation but marked by elevated creatine phosphokinase (CPK).
- NAM is associated with connective tissue diseases (CTDs), statin use, malignancies, and often occurs idiomatically.
Purpose of the Study:
- To detail the clinicopathologic features of muscle biopsy-proven NAM cases.
- To highlight the diagnostic significance of laboratory parameters like CPK levels and myositis profiles in NAM.
Main Methods:
- Retrospective analysis of 15 NAM cases diagnosed via muscle biopsy over two years.
- Review of biopsy slides, clinical data, electromyography findings, CPK levels, and myositis profiles.
Main Results:
- NAM constituted 13.63% of inflammatory myopathies, presenting with proximal muscle weakness and elevated CPK.
- Muscle biopsies revealed fiber necrosis without inflammation; anti-HMGCR and anti-SRP antibodies were positive in some patients.
- Immunotherapy yielded improvement in some patients, but two cases resulted in mortality.
Conclusions:
- Necrotizing autoimmune myopathy is a novel entity within the IIM spectrum requiring clinicopathologic correlation for diagnosis.
- NAM may be refractory to corticosteroid monotherapy, with a variable disease course including potential rapid worsening and mortality.
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