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Background <p>Sjögren’s syndrome (SjS) is a&#xa0;systemic autoimmune disease classified among the group of connective tissue diseases that is characterized not only by classical sicca symptoms but also by extraglandular manifestations. Muscular involvement is rare; however, several case reports and studies have described an association between SjS and inclusion body myositis (IBM). Polymyositis with mitochondrial pathology (PM-Mito) is another myositis subtype linked to SjS, featuring mitochondrial changes and often considered part of the IBM spectrum.</p> Case report <p>We report the case of a&#xa0;90-year-old female patient who presented with progressive dysphagia, weight loss and ultimately a&#xa0;progressive limitation in walking. During the diagnostic work-up, a&#xa0;primary SjS was diagnosed and a&#xa0;muscle biopsy enabled the diagnosis of PM-Mito.</p> Conclusion <p>This case report highlights the importance of considering neuromuscular involvement as a&#xa0;differential diagnosis in patients with SjS. The comorbid occurrence of myositis with mitochondrial pathology (PM-Mito or IBM) and SjS supports ongoing discussions about similar pathomechanistic aspects. Additionally, the report underlines the crucial role of a histopathological evaluation in cases of initially unclear myopathy as the key element for a correct diagnosis.</p>

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„Not just frailty“ – Sjögren-Syndrom und Polymyositis mit mitochondrialer Pathologie

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2025年8月22日

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  • Phillip Kremer,
  • Simon Melderis,
  • Jakob Matschke,
  • Werner Stenzel,
  • Ina Kötter,
  • Martin Krusche,
  • Marie-Therese Holzer

摘要

Background

Sjögren’s syndrome (SjS) is a systemic autoimmune disease classified among the group of connective tissue diseases that is characterized not only by classical sicca symptoms but also by extraglandular manifestations. Muscular involvement is rare; however, several case reports and studies have described an association between SjS and inclusion body myositis (IBM). Polymyositis with mitochondrial pathology (PM-Mito) is another myositis subtype linked to SjS, featuring mitochondrial changes and often considered part of the IBM spectrum.

Case report

We report the case of a 90-year-old female patient who presented with progressive dysphagia, weight loss and ultimately a progressive limitation in walking. During the diagnostic work-up, a primary SjS was diagnosed and a muscle biopsy enabled the diagnosis of PM-Mito.

Conclusion

This case report highlights the importance of considering neuromuscular involvement as a differential diagnosis in patients with SjS. The comorbid occurrence of myositis with mitochondrial pathology (PM-Mito or IBM) and SjS supports ongoing discussions about similar pathomechanistic aspects. Additionally, the report underlines the crucial role of a histopathological evaluation in cases of initially unclear myopathy as the key element for a correct diagnosis.