Clinical and histological features of immune-mediated necrotising myopathy: A multi-centre South Australian cohort study.
Neuromuscul Disord
; 30(3): 186-199, 2020 03.
Article
en En
| MEDLINE
| ID: mdl-32229165
ABSTRACT
Immune-mediated necrotising myopathy (IMNM) is a recently described entity. We describe a cohort of South Australian IMNM patients in order to define the spectrum of disease, characterise features that distinguish IMNM from other idiopathic inflammatory myopathy (IIM) subtypes and identify factors associated with clinically severe disease. Subjects were identified from the South Australian Myositis Database (SAMD), a histologically defined registry. Consecutive muscle sections from patients with IMNM (nâ¯=â¯62), other forms of IIM (nâ¯=â¯60) and histologically normal muscle (nâ¯=â¯17) were stained using immunohistochemistry and graded. Clinical information was collected from the SAMD and through retrospective chart review. IMNM patients displayed clinical and histological heterogeneity. While most (67%) were profoundly weak at presentation, 24% exhibited mild to moderate weakness and 9% had normal power. Histological myonecrosis ranged from minor to florid. The amount of myofibre complement deposition was closely associated with clinical severity. Patients of Aboriginal and Torres Strait Islander heritage and those with anti-SRP autoantibodies present with a severe phenotype. Despite intense immunotherapy, few IMNM patients recovered full power at one year follow up. The identification of clinical, serological and histological features which are associated with severe forms of the disease may have diagnostic and therapeutic utility.
Palabras clave
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Enfermedades Autoinmunes
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Sistema de Registros
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Nativos de Hawái y Otras Islas del Pacífico
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Miositis
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Necrosis
Tipo de estudio:
Etiology_studies
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
País/Región como asunto:
Oceania
Idioma:
En
Revista:
Neuromuscul Disord
Asunto de la revista:
NEUROLOGIA
Año:
2020
Tipo del documento:
Article