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Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Neurol+Neuroimmunol+Neuroinflamm 2015 ; 2 (4): ä Nephropedia Template TP
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Myopathy with anti-HMGCR antibodies: Perimysium and myofiber pathology #MMPMID26090508
Alshehri A; Choksi R; Bucelli R; Pestronk A
Neurol Neuroimmunol Neuroinflamm 2015[Aug]; 2 (4): ä PMID26090508show ga
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.