Sjögren's syndrome: autoimmune epithelitis

Sjögren's syndrome (SS) is a chronic autoimmune disorder of the exocrine glands of unknown aetiology, which is typically associated with focal lymphocytic infiltrates of glandular tissues and autoantibody responses against the Ro(SSA) and La(SSB) ribonucleoproteins. In almost one-third of patients disease involves various extraglandular sites, whereas approximately 5% of patients may also develop malignant B-cell lymphoma. In addition, features of SS are frequently encountered (5-20%) in patients with several other autoimmune rheumatic diseases, and in several respects these 'secondary' forms may be distinct from SS found alone (primary-SS), as well as from each other. The correct diagnosis and management of SS may require consideration from various specialists. Differential diagnosis includes adverse effects of drugs, sarcoidosis, lipoproteinaemias, age-related atrophy, chronic graft-versus-host disease, lymphomas, amyloidosis and infection by human immunodeficiency virus or hepatitis C virus. Based on the sequential application of the validated European classification criteria for SS, a practical algorithm for diagnosis is presented. Despite progress in the understanding of the broad clinicopathological spectrum of the entity, its treatment remains largely empirical and symptomatic. To date, the decision for systemic therapeutic intervention is primarily based on the severity of extraglandular manifestations.