Advances in Rheumatology (Dec 2019)

Recommendations from the Brazilian society of rheumatology for the diagnosis of Sjögren’s syndrome (Part I): glandular manifestations (systematic review)

  • Virginia Fernandes Moça Trevisani,
  • Sandra Gofinet Pasoto,
  • Marilena Leal Mesquita Silvestre Fernandes,
  • Maria Lúcia Lemos Lopes,
  • Sonia Cristina de Magalhães Souza Fialho,
  • Aysa César Pinheiro,
  • Laura Caldas dos Santos,
  • Simone Appenzeller,
  • Tania Fidelix,
  • Sandra Lúcia Euzébio Ribeiro,
  • Danielle Christinne Soares Egypto de Brito,
  • Tatiana Libório,
  • Maria Carmen Lopes Ferreira Silva Santos,
  • Leandro Tanure,
  • Juliana D Agostino Gennari,
  • Vinicius Tassoni Civile,
  • Ana Carolina Pereira Nunes Pinto,
  • Fabíola Reis Oliveira,
  • Jaqueline Martins de Sousa,
  • Samira Tatiyama Miyamoto,
  • Valeria Valim

DOI
https://doi.org/10.1186/s42358-019-0102-8
Journal volume & issue
Vol. 59, no. 1
pp. 1 – 17

Abstract

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Abstract Background Primary Sjögren’s syndrome (pSS) is a systemic immune-mediated disease whose main characteristic is exocrine gland inflammation and, subsequent reduction in tear and saliva production. A delayed diagnosis is common due to the nonspecific clinical manifestations of disease. The aim of the present study was to develop recommendations for the diagnosis of glandular manifestations of pSS based on evidence and expert opinion. Main body of the abstract We conducted a systematic literature review to retrieve the best evidence available on the accuracy of diagnostic tests for pSS. We also held two in-person meetings with experts (rheumatologists, pathologists, ophthalmologists and dentists) to establish their level of agreement using the Delphi method. Ultimately, we generated 18 recommendations that aim to facilitate the diagnosis of the glandular manifestations of pSS. Conclusion The diagnosis of glandular manifestations of pSS is complex and multidisciplinary. It requires specific knowledge in the field of ophthalmology, immunology, pathology and imaging, making it compulsory for the rheumatologist to work with professionals from these different areas in order to improve accuracy and early diagnosis. Glandular dysfunction tests, ANA, RF, Anti-Ro, protein electrophoresis, urinalysis, blood count, C-Reactive protein, complement, testing for syphilis and viruses (HCV, HIV) and SGUS should be investigated when dryness or systemic manifestation are present. Minor salivary gland biopsy is recommended for all anti-Ro negative or incomplete criteria cases.

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