RT Journal Article
SR Electronic
T1 Myopathy with anti-HMGCR antibodies
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP e124
DO 10.1212/NXI.0000000000000124
VO 2
IS 4
A1 Alshehri, Ali
A1 Choksi, Rati
A1 Bucelli, Robert
A1 Pestronk, Alan
YR 2015
UL http://nn.neurology.org/content/2/4/e124.abstract
AB 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.CK=creatine kinase; HMGCR=3-hydroxy-3-methylglutaryl-coenzyme A reductase; IIM=immune and inflammatory myopathy; IMPP=immune myopathies with perimysial pathology; MHC=major histocompatibility complex; NGS=normal goat serum; PBS=phosphate-buffered saline