Abstract | OBJECTIVE: 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.
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Authors | Ali Alshehri, Rati Choksi, Robert Bucelli, Alan Pestronk |
Journal | Neurology(R) neuroimmunology & neuroinflammation
(Neurol Neuroimmunol Neuroinflamm)
Vol. 2
Issue 4
Pg. e124
(Aug 2015)
ISSN: 2332-7812 [Print] United States |
PMID | 26090508
(Publication Type: Journal Article)
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