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Anti-HMGCR antibodies and asymptomatic hyperCKemia. A case report

Authors :
Torri, F.
Ali, G.
Chico, L.
Siciliano, G.
Ricci, G.
Source :
Acta Myol
Publication Year :
2021
Publisher :
Pacini Editore Srl, 2021.

Abstract

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) related myositis is a form of immune-mediated necrotizing myopathy (IMNM). Anti-HMGCR autoantibodies target HMGCR, a glycoprotein linked to the endoplasmic reticulum implied in the cholesterol synthesis pathway, and exert a pathogenic effect on skeletal muscle cells. More than 60% of patients affected by HMGCR-related myositis shares statin-exposure in their medical history. Patients commonly experience CK levels elevation, myalgia, muscle weakness and soreness at variable extent, which manifest acutely or sub acutely with a progressively worsening course, in some cases mimicking limb-girdle muscular dystrophies (LGMD) phenotype and treatment is based on an immunosuppressive strategy. Here we present the peculiar case of a previously statins-exposed 72 y.o. asymptomatic man with persistent moderate hyperCKemia and high levels of anti-HMGCR, in which pharmacotherapy has not been initiated yet, while a wait-and-see approach has been adopted instead.

Details

Language :
English
Database :
OpenAIRE
Journal :
Acta Myol
Accession number :
edsair.pmid.dedup....4bb3822cd6a188c3759039c7e200d6ff