Open AccessImmune-mediated necrotising myopathy in asymptomatic patients with high creatine kinase
Author(s) -
Izadora Fonseca Zaiden Soares,
Victoria Fernandez Comprido,
Bianca Raquel Ruoh Harn Scovoli Hsu,
Alzira Alves de Siqueira Carvalho
Publication year - 2020
Publication title -
bmj case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.231
H-Index - 26
ISSN - 1757-790X
DOI - 10.1136/bcr-2020-235457
Subject(s) - medicine , asymptomatic , myopathy , muscle biopsy , creatine kinase , weakness , inflammatory myopathy , immune system , muscle weakness , biopsy , pathology , gastroenterology , immunology , surgery
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.