Sjogren's syndrome: autoimmune epithelitis

Sjogren's syndrome (SS) is a chronic autoimmune disorder of the exocrine gl ands of unknown aetiology, which is typically associated with focal]lymphoc ytic infiltrates of glandular tissues and autoantibody responses against th e Ro(SSA) and La(SSB) ribonucleoproteins. In almost one-third of patients d isease involves various extraglandular sites, whereas approximately 5% of p atients may also develop malignant B-cell lymphoma. In addition, features o f SS are frequently encountered (5-20%) in patients with several other auto immune rheumatic diseases, and in several respects these 'secondary' forms may be distinct from SS found alone (primary-SS), as well as from each othe r. The correct diagnosis and management of SS may require consideration fro m various specialists. Differential diagnosis includes adverse effects of d rugs, sarcoidosis, lipoproteinaemias, age-related atrophy, chronic graft-ve rsus-host disease, lymphomas, amyloidosis and infection by human immunodefi ciency virus or hepatitis C virus. Based on the sequential application of t he validated European classification criteria for SS, a practical algorithm for diagnosis is presented. Despite progress in the understanding of the b road clinicopathological spectrum of the entity, its treatment remains larg ely empirical and symptomatic. To date, the decision for systemic therapeut ic intervention is primarily based on the severity of extraglandular manife stations.