Sjögren syndrome

Abstract

Sjögren syndrome is a chronic autoimmune disorder of the exocrine glands with associated lymphocytic infiltration of the affected glands. The exocrinopathy can be encountered alone (primary Sjögren syndrome or in association with other autoimmune disorders, the three most common ones being rheumatoid arthritis, systemic lupus erythematosus, and progressive systemic sclerosis (secondary Sjögren syndrome). A revised international consensus has been designed based on symptoms and objective signs. Recent studies have broadened our understanding of the etiopathogenesis and immunopathology of primary Sjögren syndrome. Systemic therapy includes treatment of the underlying systemic disorder with steroidal and nonsteroidal agents, disease-modifying agents, and cytotoxic therapy to address the extra glandular manifestations. Medical treatment of dry eye includes aqueous enhancement therapy, anti-inflammatory therapy, and secretagogues. The surgical treatment of dry eye includes punctal occlusion, tarsorrhaphy, and botulinum toxin-induced ptosis. This review highlights recent advances in understanding the underlying mechanisms of the disease as well as the therapeutic options.