Polymyositis with cytochrome C oxidase negative fibers¡ªa pathological and clinical challenge

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• 作者：Timo Siepmann ; MD^a ; ^{timo.siepmann@uniklinikum-dresden.de} ; Manfred Tesch ; MD^b ; Florian Krause ; BA^b ; Ben Min-Woo Illigens ; MD^c ; Gisela Stoltenburg-Didinger ; MD^d
• 关键词：Polymyositis ; COX-negative fibers ; Cytochrome C oxidase ; Inclusion body myositis ; Poor responsiveness to glucocorticoids
• 刊名：Annals of Diagnostic Pathology
• 出版年：2013
• 出版时间：April, 2013
• 年：2013
• 卷：17
• 期：2
• 页码：183-186
• 全文大小：1230 K

文摘

Polymyositis (PM) with cytochrome C oxidase negative fibers also referred to as PM with mitochondrial pathology (PM-Mito) is characterized by the symptoms of inclusion body myositis (IBM) and by the myopathological findings of PM except for an increase of muscle fibers with insufficient mitochondrial cytochrome C oxidase activity. Few PM-Mito cases are published; mitochondrial ultrastructure has not been studied in these patients. We report 2 PM-Mito patients with later onset than usually seen in IBM and poor responsiveness to glucocorticoids. Electron microscopy of muscle fibers showed irregular mitochondrial ultrastructure. Sj?gren syndrome related antinuclear antibodies (Anti-Ro and Anti-La) were found in one of the two patients but the typical clinical symptoms of Sj?gren syndrome such as xerostomia and keratoconjunctivitis were absent in this patient. Taken together, our observations, viewed in conjunction with the current literature, suggest that PM-Mito is an underdiagnosed disease with a multifactorial pathogenesis that should be elucidated in further studies. We want to encourage clinicians and pathologists to consider the possibility of PM-Mito in patients with atypical PM or sIBM.