title: Myopathy with anti-HMGCR antibodies: Perimysium and myofiber pathology.
abstract: OBJECTIVE
  To analyze clinical features and myopathology changes in muscle fibers, connective tissue, and vessels in 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibody-associated myopathies.
  
  
  METHODS
  Retrospective review of records and myopathologic features of 49 consecutive patients with myopathies and serum HMGCR antibodies.
  
  
  RESULTS
  Clinical features included onset age from 12 to 83 years, female predominance (67%), proximal, symmetric weakness (84%), muscle discomfort (78%), dysphagia (35%), systemic features, including skin rash and interstitial lung disease (37%), statin use (38%), and a high serum creatine kinase (83%). Myopathology included muscle fiber necrosis or regeneration (66%), myonuclear pathology (43%), perimysial connective tissue damage (61%), and lymphocytic foci (27%).
  
  
  CONCLUSIONS
  Patients with HMGCR antibody-associated myopathies present with weakness and muscle discomfort and often have damage to both perimysial connective tissue and muscle fibers, with necrosis and myonuclear pathology. Only a minority of patients with HMGCR antibody-associated myopathies have a history of statin exposure.
authors.affiliation: Department of Neurology (A.A., R.C., R.B., A.P.) and Department of Pathology and Immunology (A.P.), Washington University School of Medicine, Saint Louis, MO.
authors.forename: Ali
authors.initials: A
authors.lastname: Alshehri
authors.affiliation: Department of Neurology (A.A., R.C., R.B., A.P.) and Department of Pathology and Immunology (A.P.), Washington University School of Medicine, Saint Louis, MO.
authors.forename: Rati
authors.initials: R
authors.lastname: Choksi
authors.affiliation: Department of Neurology (A.A., R.C., R.B., A.P.) and Department of Pathology and Immunology (A.P.), Washington University School of Medicine, Saint Louis, MO.
authors.forename: Robert
authors.initials: R
authors.lastname: Bucelli
authors.affiliation: Department of Neurology (A.A., R.C., R.B., A.P.) and Department of Pathology and Immunology (A.P.), Washington University School of Medicine, Saint Louis, MO.
authors.forename: Alan
authors.initials: A
authors.lastname: Pestronk