Sjögren's syndrome

Sjögren's syndrome is a common, chronic, multisystem autoimmune disease characterized by inflammation of exocrine glands, which leads to impaired function. It is often underdiagnosed and undertreated and causes considerable morbidity. Its main clinical features are keratoconjunctivitis sicca and xerostomia. It can also involve other organ systems and can have many systemic manifestations. Patients with Sjögren's syndrome are at increased risk of lymphomas and some specific clinical features may predict lymphomas. There are now widely accepted diagnostic criteria which are helpful for both clinical management and research. A number of topical therapies and secretagogues such as pilocarpine and cevimeline relieve dryness symptoms. Immunomodulatory drugs such as hydroxychloroquine and immunosuppressive drugs are also used for treatment.