Sjögren’s syndrome is an autoimmune disease in which the body’s immune system mistakenly attacks its own moisture producing glands. As a result, a person with this condition usually experiences dry eyes and a dry mouth. Sjögren’s syndrome also may cause dryness of the skin or vagina, and rarely may affect other organs, such as the lungs, kidneys, gastrointestinal tract or nervous system.
Many patients are able to treat problems symptomatically. Others are forced to cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands, hoarseness, and difficulty in swallowing and eating. Debilitating fatigue and joint pain can seriously impair quality of life. Some patients can develop renal involvement (autoimmune tubulointerstitial nephritis) leading to proteinuria, urinary concentrating defect and distal renal tubular acidosis.
Affecting as many as four million North Americans, Sjögren’s syndrome is one of the most common of the autoimmune diseases. Nine out of 10 patients are women, and onset is typically in middle age. The cause of Sjögren’s syndrome is unknown, although heredity, infection and hormones may be contributing factors. Sjögren’s syndrome can occur by itself, called primary Sjögren’s syndrome. It also can occur with another rheumatic disease — such as rheumatoid arthritis, systemic lupus, scleroderma and polymyositis-dermatomyositis — called secondary Sjögren’s syndrome.
There is neither a known cure for Sjögren’s syndrome nor a specific treatment to permanently restore gland secretion. Instead, treatment for Sjögren’s syndrome focuses on alleviating its symptoms, often with over-the-counter medications. These include non-steroidal anti-inflammatory drugs (NSAIDs), preservative-free artificial tears, artificial salivas, unscented skin lotions, saline nasal sprays and vaginal lubricants. Exercise can help lessen joint pain and build physical stamina.
Sjögren’s can damage vital organs of the body with symptoms that may plateau or worsen, but the disease does not go into remission as with other autoimmune diseases. Some people may experience only the mild symptoms of dry eyes and mouth, while others have symptoms of severe disease.
Patients with Sjögren’s syndrome have a higher rate of non-Hodgkin lymphoma compared to both patients with other autoimmune diseases and healthy people. About 5% of patients with Sjögren’s syndrome will develop some form of lymphoid malignancy. Patients with severe cases are much more likely to develop lymphomas than patients with mild or moderate cases.