An overview of the clinical manifestations and management of primary Sjögren's syndrome

Primary Sjögren's syndrome (PSS) is a multi-system, immunemediated,condition of unknown aetiology characterized by dryness of theeyes and mouth, pain and disabling fatigue. Other organs such as the skin,the nervous system, the lungs and the kidneys can also be affected. PSS isalso associated with a markedly increased risk of lymphomadevelopment. The health-related quality of life of patients with PSS issignificantly impaired and the direct and indirect health economic costassociated with the condition is substantial.The true prevalence of PSS is unknown but is estimated to bebetween 0.1-0.4% of the adult population with a strong female bias. Thediagnosis is often delayed with PSS patients presenting to their doctorsafter suffering from the symptoms for many years. Improved linksbetween rheumatologists, ophthalmologists, oral medicine specialists,dentists, opticians, and general practitioners can facilitate early diagnosisand reduce risk of long-term disability.The American European Consensus Group (AECG) classificationcriteria 2002 are the current gold standard for the classification of PSS,although provisional American College of Rheumatology (ACR) criteriahave recently been published. The development of consensus criteria bythe ACR-European League Against Rheumatism (EULAR) workinggroup to bring together the two criteria is in progress. Additionally,salivary gland ultrasound may also be an important component of thediagnostic criteria in the near future. Salivary gland sialometry,sialochemistry, tear osmolarity may be useful adjuncts, and the discoveryof new diagnostic biomarkers is an area of active research.Optimal assessment of patients with PSS, should encompass not onlyobjective parameters such as abnormalities in blood tests and changes intear and salivary flow, but also patient-reported outcome measures andimpact on quality of life. Symptoms of PSS are often non-specific,therefore these symptoms must be actively explored. The recentdevelopment of EULAR outcome measurement tools for the assessmentof systemic disease activity and patient-reported outcome has greatlyfacilitated standardised assessment of PSS in clinics and in research.Management of PSS can be broadly classified into interventionstargeting the glandular or systemic manifestations of the disease.Evidence-based strategies in PSS management remain elusive, instead,treatments are often based on empirical evidence or experience of thetreating clinicians. The effective management of fatigue remains a majorchallenge. Recent advances in the understanding of PSS pathogenesishave led to the study of several biological therapies for the PSS withsome encouraging data.