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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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case presentation<\/jats:title><jats:p>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 \u03bcmol\/minute\/g tissue; normal range 1.03 to 3.83 \u03bcmol\/minute\/g tissue), elevated acid maltase activity (23.39 \u03bcmol\/minute\/g tissue; normal range 1.74 to 9.98 \u03bcmol\/minute\/g tissue) and elevated neutral maltase activity (35.89 \u03bcmol\/minute\/g tissue; normal range 4.35 to 16.03 \u03bcmol\/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, \u03b1-lipoic acid and ribose resulted in dramatic clinical improvement.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec>","DOI":"10.1186\/1752-1947-5-262","type":"journal-article","created":{"date-parts":[[2011,8,3]],"date-time":"2011-08-03T06:19:37Z","timestamp":1312352377000},"update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Metabolic myopathy presenting with polyarteritis nodosa: a case report"],"prefix":"10.1186","volume":"5","author":[{"given":"Sahana","family":"Vishwanath","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mishal","family":"Abdullah","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amro","family":"Elbalkhi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"suffix":"Jr","given":"Julian L","family":"Ambrus","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2011,6,30]]},"reference":[{"key":"1652_CR1","doi-asserted-by":"publisher","first-page":"118","DOI":"10.1007\/s11910-010-0096-4","volume":"10","author":"A Berardo","year":"2010","unstructured":"Berardo A, DiMauro S, Hirano M: A diagnostic algorithm for metabolic myopathies. 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