Sjögren's syndrome is the next most common autoimmune disorder after rheumatoid arthritis. It results from a chronic exocrine gland dysfunction and is marked by diminished lacrimal and salivary gland secretion (sicca complex). The syndrome affects more women (about 90%) than men. The mean age of occurrence is 50.
Sjögren's syndrome may be a primary disorder, or it may be associated with inflammatory connective tissue disorders, such as rheumatoid arthritis (about 50% of patients), scleroderma, systemic lupus erythematosus, primary biliary cirrhosis, Hashimoto's thyroiditis, polyarteritis, and interstitial pulmonary fibrosis.
Nephritis (seldom leading to chronic renal failure) may affect up to 40% of patients with primary Sjögren's syndrome and can result in renal tubular acidosis in about 25% of patients.
Patients with Sjögren's syndrome may also have Raynaud's phenomenon (about 20%) and vasculitis (usually limited to the skin and may be systemic or localized in the legs). Sensory polyneuropathy and biochemical hypothyroidism (resembling Hashimoto's thyroiditis) occur in up to 50% of patients. Rarely, systemic necrotizing vasculitis develops and involves the skin, peripheral nerves, and GI tract.
Overall, the prognosis for a patient with Sjögren's syndrome is good.
No one knows what causes Sjögren's syndrome, but researchers think that genetic and environmental factors may contribute to its development. Viral or bacterial infection - or possibly exposure to pollen - may trigger this disease in a genetically susceptible person. Tissue damage results from infiltration by lymphocytes or deposition of immune complexes. Lymphocytic infiltration may be classified as benign lymphoma, malignant lymphoma, or pseudolymphoma (nonmalignant but tumor like aggregates of lymphoid cells).
Diagnosis of Sjögren's syndrome is based on the patient having at least three consecutive months of bothersome eye and/or mouth dryness. A variety of tests can then be done to determine the quantity of tears produced, the quantity of saliva produced, and the presence or absence of antibodies that could be involved in the destruction of glands.
The goal of treatment is to relieve the patient's symptoms. Treatment includes conservative measures to moisten the eyes and mouth. Artificial tears and sustained-release cellulose capsules help to relieve ocular dryness. If an eye infection develops, the patient receives antibiotics; topical steroids should be avoided.
Mouth dryness can be relieved by using a methylcellulose swab or spray and by drinking plenty of fluids, especially at mealtime. New agents for treatment of saliva hypofunction, such as pilocarpine hydrochloride and bromnetine, may be useful. Meticulous oral hygiene includes regular flossing, brushing, fluoride treatments, and frequent dental checkups.
Other treatment measures vary according to extraglandular effects. For parotid gland enlargement, treatment involves local heat and analgesia; for arthritis and arthralgias, hydroxychloroquine or nonsteroidal anti-inflammatory drugs. For interstitial pulmonary and renal disease, treatment relies on corticosteroids; for cutaneous vasculitis, corticosteroids are less effective.
For the patient with lymphoma, treatment includes a combination of chemotherapy, surgery, and radiation therapy.
Since the cause of Sjögren's syndrome is unknown, there are no known ways to prevent this syndrome.
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