Kremer Phillip, Melderis Simon, Matschke Jakob, Stenzel Werner, Kötter Ina, Krusche Martin, Holzer Marie-Therese
III. Medizinische Klinik, Sektion für Rheumatologie und entzündliche Systemerkrankungen, Universitätsklinikum Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Deutschland.
Institut für Neuropathologie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Deutschland.
Z Rheumatol. 2025 Aug 22. doi: 10.1007/s00393-025-01702-3.
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.
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.
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.
干燥综合征（SjS）是一种归类于结缔组织病的全身性自身免疫性疾病，其不仅以典型的干燥症状为特征，还具有腺体外表现。肌肉受累较为罕见；然而，一些病例报告和研究描述了SjS与包涵体肌炎（IBM）之间的关联。伴有线粒体病理改变的多发性肌炎（PM-Mito）是另一种与SjS相关的肌炎亚型，其具有线粒体改变，常被认为是IBM谱系的一部分。
我们报告一例90岁女性患者，其表现为进行性吞咽困难、体重减轻，最终出现进行性行走受限。在诊断检查过程中，诊断出原发性SjS，肌肉活检确诊为PM-Mito。
本病例报告强调了在SjS患者中将神经肌肉受累作为鉴别诊断的重要性。肌炎合并线粒体病理改变（PM-Mito或IBM）与SjS的共病现象支持了关于类似发病机制方面的持续讨论。此外，该报告强调了在初始不明原因肌病病例中组织病理学评估作为正确诊断关键要素的重要作用。
