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Myopathy with anti-HMGCR antibodies: Perimysium and myofiber pathology
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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Lippincott Williams & Wilkins
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4459046/ https://www.ncbi.nlm.nih.gov/pubmed/26090508 http://dx.doi.org/10.1212/NXI.0000000000000124 |
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author | Alshehri, Ali Choksi, Rati Bucelli, Robert Pestronk, Alan |
author_facet | Alshehri, Ali Choksi, Rati Bucelli, Robert Pestronk, Alan |
author_sort | Alshehri, Ali |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-4459046 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Lippincott Williams & Wilkins |
record_format | MEDLINE/PubMed |
spelling | pubmed-44590462015-06-18 Myopathy with anti-HMGCR antibodies: Perimysium and myofiber pathology Alshehri, Ali Choksi, Rati Bucelli, Robert Pestronk, Alan Neurol Neuroimmunol Neuroinflamm Article 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. Lippincott Williams & Wilkins 2015-06-04 /pmc/articles/PMC4459046/ /pubmed/26090508 http://dx.doi.org/10.1212/NXI.0000000000000124 Text en © 2015 American Academy of Neurology This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND) (http://creativecommons.org/licenses/by-nc-nd/4.0/) , which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially. |
spellingShingle | Article Alshehri, Ali Choksi, Rati Bucelli, Robert Pestronk, Alan Myopathy with anti-HMGCR antibodies: Perimysium and myofiber pathology |
title | Myopathy with anti-HMGCR antibodies: Perimysium and myofiber pathology |
title_full | Myopathy with anti-HMGCR antibodies: Perimysium and myofiber pathology |
title_fullStr | Myopathy with anti-HMGCR antibodies: Perimysium and myofiber pathology |
title_full_unstemmed | Myopathy with anti-HMGCR antibodies: Perimysium and myofiber pathology |
title_short | Myopathy with anti-HMGCR antibodies: Perimysium and myofiber pathology |
title_sort | myopathy with anti-hmgcr antibodies: perimysium and myofiber pathology |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4459046/ https://www.ncbi.nlm.nih.gov/pubmed/26090508 http://dx.doi.org/10.1212/NXI.0000000000000124 |
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