title: [Anti-HMG-CoA reductase necrotizing myopathy: A report of 4 cases].
abstract: BACKGROUND
  Anti-3-hydroxy-3-méthylglutaryl-coenzyme A reductase antibody-associated necrotizing autoimmune myopathy has been recently described (2011). This myopathy is distinct from statin toxic myopathy. Our objective is to report on the clinical and para-clinical characteristics of this myopathy and to show the difficulties of therapeutic care.
  
  
  METHODS
  We describe 4 cases of patients followed-up in Brittany, France. All data have been analyzed retrospectively. The mean age of our patients was 59.5 years, with a sex ratio of 1. The clinical presentation was homogeneous, with a subacute painful proximal and symmetrical weakness, without extra-muscular involvement. Other presentations have been described (including pseudo-dystrophic presentation). All patients had a previous statin medication (mean duration of 3.75 years) although this criteria is not a requisite. All patients had high levels of creatine kinase and abnormal electromyographic examination. The pathological pattern on muscular biopsy was a necrotizing myopathy without significant inflammatory cells infiltration. Cardio-respiratory function was normal and no associated neoplasia was found. Over the follow-up, we observed a marked corticosteroid-dependence, not improved by immunosuppressive drugs (azathioprine and methotrexate). The benefit of intravenous immunoglobulin was clear with, sometimes, prolonged responses.
  
  
  CONCLUSIONS
  An early diagnosis of this myopathy is necessary in order to introduce an immunotherapy associated with a close monitoring. The therapeutic strategy (within which the stead of intravenous immunoglobulin seems increased) remains to be defined and long-term prospective studies are thus needed.
authors.affiliation: Service de neurologie, centre hospitalier Yves-le-Foll, 10, rue Marcel-Proust, 22027 Saint-Brieuc cedex 1, France. Electronic address: martijulien@yahoo.fr.
authors.forename: J
authors.initials: J
authors.lastname: Marti
authors.affiliation: Service de neurologie, centre hospitalier Yves-le-Foll, 10, rue Marcel-Proust, 22027 Saint-Brieuc cedex 1, France.
authors.forename: I
authors.initials: I
authors.lastname: Kolev
authors.affiliation: Service de médecine interne, hôpital du Scorff, centre hospitalier de Bretagne Sud, 5, avenue du Choiseul, 56100 Lorient, France.
authors.forename: A
authors.initials: A
authors.lastname: Lorleac'h
authors.affiliation: Service de médecine interne, hôpital privé Sévigné, 3, rue du Chêne-Germain, 35576 Cesson-Sévigné cedex, France.
authors.forename: S
authors.initials: S
authors.lastname: Besnard
authors.affiliation: Service de neurologie, centre hospitalier Yves-le-Foll, 10, rue Marcel-Proust, 22027 Saint-Brieuc cedex 1, France.
authors.forename: M
authors.initials: M
authors.lastname: Pihan
authors.affiliation: Service d'anatomie et cytologie pathologiques, hôpital Morvan, centre hospitalier régional et universitaire de Brest, 2, avenue Foch, 29609 Brest cedex, France.
authors.forename: P
authors.initials: P
authors.lastname: Marcorelles
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000009:adverse effects* / Q000097:blood; D001327:Autoimmune Diseases / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D005260:Female; D005602:France; D006801:Humans; D006903:Hydroxymethylglutaryl CoA Reductases / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology*; D009135:Muscular Diseases / Q000150:complications / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D009336:Necrosis / Q000150:complications; D012189:Retrospective Studies
chemical_list: D001323:Autoantibodies; D006903:Hydroxymethylglutaryl CoA Reductases
keywords: Anti-HMGCR; Immune-mediated necrotizing myopathy; Myopathie nécrosante auto-immune; Statines; Statins