Sjögren's syndrome is both an organ-specific and a systemic autoimmune disease. Patients with dry eyes and dry mouth usually receive symptomatic treatment. Topical cyclosporine A may be administered to patients with dry eyes. The efficacy of muscarinic agonists, such as pilocarpine and cevimeline, in increasing salivary and lacrimal flow and improving patient symptoms has been well documented. Mild immunomodulatory or immunosuppressive medication is preferable to prevent the mild progressive course of the disorders. Other patients who show aggressive progression to organ disorders should be managed differently. High doses of corticosteroids, immunosuppressive agents or new biological agents, such as antibodies to CD20, are required for these patients. The elimination of B lymphocytes by anti-CD20 antibodies may be highly beneficial; however, the development of more selective drugs based on the pathogenesis of the disease is required for the future treatment of Sjögren's syndrome.