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Sjögren syndrome associated with protein-losing enteropathy: case-based review

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Abstract

The association between Sjögren’s syndrome (SS) and protein-losing enteropathy (PLE) was scarcly reported. To analyze the clinical, therapeutic, and outcome characteristics of patients with SS and PLE and also to delineate the potential mechanisms and pathways connecting the gut to SS targeted organ’s pathology. Systematic screening was conducted using PubMed/MEDLINE, LILACS, SciELO, Web of Science, and Cochrane, dating 1980 to 2020. SS and PLE were the key words. Eighteen patients with SS and PLE were summarized. The patient’s ages ranged between 20 and 88 years, and only 4 were males. Primary SS was observed in most cases. Anti-Ro was detected in 100% of the cases while anti-La was reported in 64% of them. The clinical manifestations were protein loss, edema of the lower limbs, pleural effusion, ascites, facial edema, anasarca, diarrhea, and weight loss. Among these clinical manifestations, edema of the lower limbs was the most severe. Albumin concentration was 0.9–3.4 g/dL which increased to 2.8–4.3 g/dL after treatment. Small bowel biopsy was performed in all of the cases. Concerning the therapy, all the patients received systemic glucocorticoids. All of them improved. The period of onset of improvement ranged from 3 weeks to 36 months (an average of 3 months). The early diagnosis and appropriate therapy of PLE in patients with anti-Ro positive SS and who present edema, anasarca, or hypoalbuminemia is vital for a beneficial outcome. An excellent clinical improvement in all the cases was observed when treated early enough by cortico-therapy, thus preventing patient’s deterioration, complications, and reducing morbidity and potential mortality.

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Funding

The work is supported by a grant from the Government of the Russian Federation (contract 14.W03.31.0009 of 13/02/2017) for state support of scientific research conducted under the supervision of leading scientists.

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Author notes

  1. Jozélio Freire de Carvalho and Aaron Lerner contributed equally to this work.

Authors and Affiliations

  1. Institute for Health Sciences from Federal University of Bahia, Rua das Violetas, 42, ap. 502, Pituba, Salvador, Bahia, Brazil

    Jozélio Freire de Carvalho

  2. Chaim Sheba Medical Center, The Zabludowicz Research Center for Autoimmune Diseases, Tel Hashomer, Israel

    Aaron Lerner & Yehuda Shoenfeld

  3. UNIFACS School of Medicine, Salvador, BA, Brazil

    Caio Marco Gonçalves

  4. Saint Petersburg State University, St. Petersburg, Russia

    Yehuda Shoenfeld

Authors
  1. Jozélio Freire de Carvalho
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  2. Aaron Lerner
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  3. Caio Marco Gonçalves
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  4. Yehuda Shoenfeld
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Contributions

CG: Literature screening, case reporting, manuscript preparation.

JFC: Conceptualization, literature screening and data curation, manuscript writing.

AL: Introduction and discussion on the gut-ss axis relationships.

YS: Review, editing, and supporting.

Corresponding author

Correspondence to Jozélio Freire de Carvalho.

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de Carvalho, J.F., Lerner, A., Gonçalves, C.M. et al. Sjögren syndrome associated with protein-losing enteropathy: case-based review. Clin Rheumatol 40, 2491–2497 (2021). https://doi.org/10.1007/s10067-020-05487-5

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  • DOI: https://doi.org/10.1007/s10067-020-05487-5

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