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Association of early-onset myasthenia gravis and primary Sjögren’s syndrome: a case-based narrative review

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A Correction to this article was published on 11 August 2022

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Abstract

Coexistent myasthenia gravis (MG) and primary Sjögren’s syndrome (pSS) is an absolute rarity. That is kind of a surprise as both entities seem to share the same corresponding immunologic mechanisms. We hereby report the case of a 41-year-old woman with coincident early-onset MG (EOMG) and pSS. Because EOMG was the leading clinical feature, she was primarily treated by innovative non-intubated uniportal subxiphoid video-assisted thoracoscopic surgery (VATS) thymectomy. As the association of EOMG and pSS is so unusual, we contextualize our findings with the relevant literature. Particular relevance is an anti-nuclear antibody screening throughout the clinical course of MG and—in reverse—a screening for MG variables when pSS patients complain either muscle fatigability or fatigable ptosis. As pSS patients do not develop any serious morbidity, supervising MG progress in patients with both diseases is of utmost importance. Apart from conscientious pSS diagnosis, prompt adjusting of EOMG progress is the essential aspect of targeted treatment. In this context, it is relevant that therapeutic decisions are made in a multidisciplinary approach. Due to its rarity, multicenter prospective studies of larger sample sizes are indispensably needed to obtain a better understanding of this unusual link.

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Data availability

The datasets generated and analyzed during the current study are available from the corresponding author on reasonable request. In principle, all data generated and analyzed during this study are included in this article.

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Authors and Affiliations

  1. Department of Thoracic Surgery, Katholisches Klinikum Koblenz-Montabaur, Rudolf-Virchow-Str. 7-9, 56073, Koblenz, Germany

    Marc Hartert & Martin Huertgen

  2. Institute of Pathology, Franz-Weis-Str. 13, 56073, Koblenz, Germany

    Balint Melcher

Authors
  1. Marc Hartert
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Contributions

All authors contributed in different degrees to the conception and design of the work, the acquisition, analysis, and interpretation of data. M. Hartert drafted the manuscript. B. Melcher created and provided the pathological images. M. Huertgen helped with finalizing the manuscript. All authors approved the final manuscript.

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Correspondence to Marc Hartert.

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The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures depicted in this case were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient for publication of this case report and accompanying images.

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The original online version of this article was revised: Table 2 data has been aligned correctly.

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Hartert, M., Melcher, B. & Huertgen, M. Association of early-onset myasthenia gravis and primary Sjögren’s syndrome: a case-based narrative review. Clin Rheumatol 41, 3237–3243 (2022). https://doi.org/10.1007/s10067-022-06294-w

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