Clinicopathological data analysis of immune-mediated necrotic diseases

Abstract

From January 1, 2017 to June 30, 2020, 14 patients diagnosed with immune-mediated necrotizing myopathy were retrospectively analyzed, and their clinical features, muscle pathology, and myositis antibody spectrum were summarized and analyzed. Most of the 14 patients had onset in middle age, were more common in women, and had an acute or subacute course. The clinical manifestations are mainly muscle weakness, which may involve the proximal muscles of the limbs, neck flexors, and masticatory muscles; it may be accompanied by multi-system involvement such as rash, oral ulcers, dry mouth, dry eyes, and interstitial lung disease. Creatine kinase moderately to severely elevated. Electromyography showed myogenic damage. Myositis antibody detection is mainly positive for anti-signal recognition particle antibodies and anti-3-hydroxy-3-methylglutaryl-CoA reductase antibodies. Muscle pathology manifests as diffuse or scattered muscle fiber necrosis, phagocytosis, and regeneration, with no or a small amount of inflammatory cell infiltration. Immunohistochemical staining showed mainly CD68+ macrophage infiltration. The clinical manifestations of immune-mediated necrotizing myopathy are complex and can involve multiple systems, requiring multidisciplinary joint diagnosis and treatment. In addition to routine auxiliary examinations, myositis antibody spectrum detection is very important in this disease and is helpful for diagnosis. [International Journal of Neurology Neurosurgery, 2021, 48(6):506-510