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Sjögren’s syndrome with adult-onset Still’s disease: an overlap syndrome?

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Abstract

Adult-onset Still’s disease (AOSD) is a rare systemic auto-inflammatory disease that is an exclusive diagnosis that needs to previously exclude infections, tumors, and rheumatic diseases. There are few reports on AOSD overlapping with other rheumatic diseases. We reported a 55-year-old male who presented with a high, daily spiking fever associated with an evanescent salmon-pink rash, arthralgia, and sore throat. He had a history of dryness of the mouth and eyes for decades with no medical treatment. On admission, tests for antinuclear antibody (ANA) and anti-SSA/Ro-52 antibody were positive, and salivary gland biopsy showed focal lymphocytic sialadenitis with a focus score of ≥ 1 foci/4 mm2, which was consistent with a diagnosis of primary Sjögren’s syndrome (SS). However, the disease activity of SS was low at the time of the report. Combined with significantly elevated acute phase reactants, the patient also met the classification criteria of both Yamakuchi and Futel for AOSD. His clinical symptoms were relieved quickly with glucocorticoid therapy. We also reviewed the literature on SS with AOSD and AOSD with other rheumatic diseases, and scattered case reports were retrieved. So we think that AOSD is not an absolutely exclusive diagnosis and can occur in patients with other rheumatic diseases. To our knowledge, this is the only literature review of a reported AOSD case in a SS patient.

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Acknowledgements

The authors thank American Journal Experts for the language editing of the manuscript.

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This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors, including pharmaceutic agencies.

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Authors and Affiliations

  1. Department of Rheumatology and Immunology, Hangzhou Normal University Affiliated Hospital, No. 126, Wenzhou Road, Hangzhou, 310015, China

    Pingyang Han, Chen Sun, Yinghong Zhang & Juzhen Yan

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  1. Pingyang Han
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  2. Chen Sun
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  3. Yinghong Zhang
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Contributions

All authors made substantial contributions to the article. PH and JY designed the work, interpreted the data, drafted the paper, and revised it. CS and YZ acquired the data, interpreted, and revised it. PH wrote the first draft of the manuscript, and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Juzhen Yan.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards. Authors fulfilled the ICMJE authorship criteria.

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Han, P., Sun, C., Zhang, Y. et al. Sjögren’s syndrome with adult-onset Still’s disease: an overlap syndrome? . Rheumatol Int 42, 1281–1285 (2022). https://doi.org/10.1007/s00296-021-04900-9

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  • DOI: https://doi.org/10.1007/s00296-021-04900-9

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