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Innovations in treatment
Case report
Immune-mediated necrotising myopathy in asymptomatic patients with high creatine kinase
  1. Izadora Fonseca Zaiden Soares,
  2. Victoria Fernandez Comprido,
  3. Bianca Raquel Ruoh Harn Scovoli Hsu and
  4. Alzira Alves de Siqueira Carvalho
  1. Neurosciences, Centro Universitário Saúde ABC, Santo Andre, São Paulo, Brazil
  1. Correspondence to Professor Alzira Alves de Siqueira Carvalho; alzirasiqueiracarvalho{at}gmail.com

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.

  • neurology
  • muscle disease

https://doi.org/10.1136/bcr-2020-235457

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    Footnotes

    • Contributors AAdSC and IFZS planned the study. VFC and BRRHSH made corrections and suggestions.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.