Sjögren syndrome: looking forward to the future

Author:

Zandonella Callegher Sara1,Giovannini Ivan2ORCID,Zenz Sabine3,Manfrè Valeria2,Stradner Martin H.3ORCID,Hocevar Alojzija4,Gutierrez Marwin56,Quartuccio Luca2,De Vita Salvatore2,Zabotti Alen7ORCID

Affiliation:

1. Department of Rheumatology, Hospital of Bruneck, Bruneck, Italy

2. Rheumatology Clinic, Department of Medicine, University of Udine, c/o Azienda Sanitaria Universitaria Friuli Centrale, Udine, Italy

3. Division of Rheumatology and Immunology, Medical University Graz, Graz, Austria

4. Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, Slovenia

5. Division of Musculoskeletal and Rheumatic Diseases, Instituto Nacional de Rehabilitacion, Mexico City, Mexico

6. Rheumatology Center of Excellence, Mexico City, Mexico

7. Rheumatology Clinic, Department of Medicine, University of Udine, c/o Azienda Sanitaria Universitaria Friuli Centrale, Piazzale Santa Maria della Misericordia 15, 33100 Udine, Italy

Abstract

Primary Sjögren’s syndrome (pSS) is a heterogeneous disease characterised by a wide spectrum of manifestations that vary according to the different stages of the disease and among different subsets of patients. The aim of this qualitative literature review is to summarise the recent advances that have been reported in pSS, ranging from the early phases to the established disease and its complications. We analysed the diagnostic, prognostic, and management aspects of pSS, with a look into future clinical and research developments. The early phases of pSS, usually antedating diagnosis, allow us to investigate the pathophysiology and risk factors of the overt disease, thus allowing better and timely patient stratification. Salivary gland ultrasound (SGUS) is emerging as a valid complementary, or even alternative, tool for histopathology in the diagnosis of pSS, due to a standardised scoring system with good agreement and performance. Other promising innovations include the application of artificial intelligence to SGUS, ultrasound-guided core needle biopsy, and a wide array of novel diagnostic and prognostic biomarkers. Stratifying pSS patients through the integration of clinical, laboratory, imaging, and histopathological data; differentiating between activity-related and damage-related manifestations; and identifying patients at higher risk of lymphoma development are essential steps for an optimal management and individualised treatment approach. As new treatment options are emerging for both glandular and systemic manifestations, there is a need for a more reliable treatment response evaluation. pSS is a complex and heterogeneous disease, and many distinct aspects should be considered in the different stages of the disease and subsets of patients. In recent years, efforts have been made to improve our understanding of the disease, and certainly in the coming years, some of these novelties will become part of our routine clinical practice, thus improving the management of pSS patients.

Publisher

SAGE Publications

Subject

Orthopedics and Sports Medicine,Rheumatology

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