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Immune-mediated necrotising myopathy in asymptomatic patients with high creatine kinase
Izadora Fonseca Zaiden Soares1, Victoria Fernandez Comprido1, Bianca Raquel Ruoh Harn Scovoli Hsu1
1Neurosciences, Centro Universitário Saúde ABC, Santo Andre, São Paulo, Brazil.
Abstract:
Subacute symmetrical proximal muscle weakness and persistent elevated creatine kinase levels are typical of immune-mediated necrotising myopathy (IMNM). These conditions are accompanied by copious myofibre necrosis, degeneration and regeneration with minimal to no inflammation on muscle biopsy. We report two cases (case 1 and case 2) of asymptomatic IMNM from different families with hyperCKaemia associated with positive anti-signal recognition particle (SRP) and anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibodies, respectively, and we also reviewed the literature. There are only a few previous descriptions of patients with asymptomatic IMNM.The disease onset could be insidious and lead to delayed diagnosis and treatment. We recommend testing for the anti-HMGCR and anti-SRP antibodies in patients with idiopathic hyperCKaemia because they could show no symptoms of this disorder.
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