Sjögren Syndrome

Sjögren syndrome, also called sicca syndrome, chronic inflammatory disorder characterized by severe dryness of the eyes and mouth that results from a diminution in secretion of tears and saliva. Dryness may also involve the nose, pharynx, larynx, and tracheobronchial tree. Approximately half the persons affected also have rheumatoid arthritis or, less commonly, some other connective tissue disease, such as scleroderma, polymyositis, or systemic lupus erythematosus. The great majority of the persons affected by Sjögren syndrome are women.

Infiltration (gradual assemblage) of white blood cells (lymphocytes) and plasma cells leads to enlargement of the parotid or other salivary glands in half the patients. There may also be enlargement of the spleen, diminution in white blood cell numbers, Raynaud syndrome, vasculitis (inflammation of blood vessels) with chronic leg ulcers, a disease of the peripheral or trigeminal nerves, chronic Hashimoto thyroiditis (inflammation of the thyroid), enlargement of the liver, and inflammation of the pancreas. Persons with chronic Sjögren syndrome are at risk of various malignancies, including reticulum cell sarcoma, non-Hodgkin lymphoma, and primary macroglobulinemia (the presence in the blood of globulins of high molecular weight).

Treatment toward relief of symptoms includes the administration of artificial tears to lessen ocular dryness. Corticosteroids, hydroxychloroquine, and other immunosuppressive drugs have been employed with some success for the more serious manifestations.