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Metabolic myopathy presenting with polyarteritis nodosa: a case report
INTRODUCTION: To the best of our knowledge, we describe for the first time a patient in whom an unusual metabolic myopathy was identified after failure to respond to curative therapy for a systemic vasculitis, polyarteritis nodosa. We hope this report will heighten awareness of common metabolic myop...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2011
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3148996/ https://www.ncbi.nlm.nih.gov/pubmed/21718511 http://dx.doi.org/10.1186/1752-1947-5-262 |
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author | Vishwanath, Sahana Abdullah, Mishal Elbalkhi, Amro Ambrus, Julian L |
author_facet | Vishwanath, Sahana Abdullah, Mishal Elbalkhi, Amro Ambrus, Julian L |
author_sort | Vishwanath, Sahana |
collection | PubMed |
description | INTRODUCTION: To the best of our knowledge, we describe for the first time a patient in whom an unusual metabolic myopathy was identified after failure to respond to curative therapy for a systemic vasculitis, polyarteritis nodosa. We hope this report will heighten awareness of common metabolic myopathies that may present later in life. It also speculates on the potential relationship between metabolic myopathy and systemic vasculitis. CASE PRESENTATION: A 78-year-old African-American woman with a two-year history of progressive fatigue and exercise intolerance presented to our facility with new skin lesions and profound muscle weakness. Skin and muscle biopsies demonstrated a medium-sized artery vasculitis consistent with polyarteritis nodosa. Biochemical studies of the muscle revealed diminished cytochrome C oxidase activity (0.78 μmol/minute/g tissue; normal range 1.03 to 3.83 μmol/minute/g tissue), elevated acid maltase activity (23.39 μmol/minute/g tissue; normal range 1.74 to 9.98 μmol/minute/g tissue) and elevated neutral maltase activity (35.89 μmol/minute/g tissue; normal range 4.35 to 16.03 μmol/minute/g tissue). Treatment for polyarteritis nodosa with prednisone and cyclophosphamide resulted in minimal symptomatic improvement. Additional management with a diet low in complex carbohydrates and ubiquinone, creatine, carnitine, folic acid, α-lipoic acid and ribose resulted in dramatic clinical improvement. CONCLUSIONS: Our patient's initial symptoms of fatigue, exercise intolerance and progressive weakness were likely related to her complex metabolic myopathy involving both the mitochondrial respiratory chain and glycogen storage pathways. Management of our patient required treatment of both the polyarteritis nodosa as well as metabolic myopathy. Metabolic myopathies are common and should be considered in any patient with exercise intolerance. Metabolic myopathies may complicate the management of various disease states. |
format | Online Article Text |
id | pubmed-3148996 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2011 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-31489962011-08-03 Metabolic myopathy presenting with polyarteritis nodosa: a case report Vishwanath, Sahana Abdullah, Mishal Elbalkhi, Amro Ambrus, Julian L J Med Case Reports Case Report INTRODUCTION: To the best of our knowledge, we describe for the first time a patient in whom an unusual metabolic myopathy was identified after failure to respond to curative therapy for a systemic vasculitis, polyarteritis nodosa. We hope this report will heighten awareness of common metabolic myopathies that may present later in life. It also speculates on the potential relationship between metabolic myopathy and systemic vasculitis. CASE PRESENTATION: A 78-year-old African-American woman with a two-year history of progressive fatigue and exercise intolerance presented to our facility with new skin lesions and profound muscle weakness. Skin and muscle biopsies demonstrated a medium-sized artery vasculitis consistent with polyarteritis nodosa. Biochemical studies of the muscle revealed diminished cytochrome C oxidase activity (0.78 μmol/minute/g tissue; normal range 1.03 to 3.83 μmol/minute/g tissue), elevated acid maltase activity (23.39 μmol/minute/g tissue; normal range 1.74 to 9.98 μmol/minute/g tissue) and elevated neutral maltase activity (35.89 μmol/minute/g tissue; normal range 4.35 to 16.03 μmol/minute/g tissue). Treatment for polyarteritis nodosa with prednisone and cyclophosphamide resulted in minimal symptomatic improvement. Additional management with a diet low in complex carbohydrates and ubiquinone, creatine, carnitine, folic acid, α-lipoic acid and ribose resulted in dramatic clinical improvement. CONCLUSIONS: Our patient's initial symptoms of fatigue, exercise intolerance and progressive weakness were likely related to her complex metabolic myopathy involving both the mitochondrial respiratory chain and glycogen storage pathways. Management of our patient required treatment of both the polyarteritis nodosa as well as metabolic myopathy. Metabolic myopathies are common and should be considered in any patient with exercise intolerance. Metabolic myopathies may complicate the management of various disease states. BioMed Central 2011-06-30 /pmc/articles/PMC3148996/ /pubmed/21718511 http://dx.doi.org/10.1186/1752-1947-5-262 Text en Copyright ©2011 Vishwanath et al; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Vishwanath, Sahana Abdullah, Mishal Elbalkhi, Amro Ambrus, Julian L Metabolic myopathy presenting with polyarteritis nodosa: a case report |
title | Metabolic myopathy presenting with polyarteritis nodosa: a case report |
title_full | Metabolic myopathy presenting with polyarteritis nodosa: a case report |
title_fullStr | Metabolic myopathy presenting with polyarteritis nodosa: a case report |
title_full_unstemmed | Metabolic myopathy presenting with polyarteritis nodosa: a case report |
title_short | Metabolic myopathy presenting with polyarteritis nodosa: a case report |
title_sort | metabolic myopathy presenting with polyarteritis nodosa: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3148996/ https://www.ncbi.nlm.nih.gov/pubmed/21718511 http://dx.doi.org/10.1186/1752-1947-5-262 |
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