Sjogren's Syndrome - Definition, Causes, Symptoms and Treatment

Sjögren’s syndrome is a chronic inflammatory disorder of probable autoimmune nature. Autoimmune diseases are characterized by the abnormal production of extra antibodies in the blood that are directed against various tissues of the body.   About 50% of the time Sjögren's syndrome occurs alone, and 50% of the time it occurs in the presence of another connective tissue disease. The classic signs of the Sjögren’s syndrome, therefore, includes enlargement of the parotid glands with mucosal dryness manifest by dry mouth (xerostomia) and dry eyes (xerophthalmia). Sjogren's syndrome that only involves gland inflammation (resulting dry eyes and mouth, etc.), and is not associated with a connective tissue disease, is referred to as primary Sjogren's syndrome. Secondary Sjogren's syndrome involves not only gland inflammation, but is also associated with a connective tissue disease, such as rheumatoid arthritis, systemic lupus erythematosus, or scleroderma. 

Symptoms associated with Sjögren's are caused by the infiltration of immune-system cells, usually B and T lymphocytes, into the glands responsible for secreting fluid. This condition can affect people of any age, but symptoms usually appear between the ages of 45 and 55. In Sjogren's syndrome, your immune system attacks the glands that make tears and saliva. Sjögren's may also cause dryness of other organs, affecting the kidneys, GI tract, blood vessels, lung, liver, pancreas, and the central nervous system. Many patients experience debilitating fatigue and joint pain. Symptoms can plateau, worsen, or go into remission. While some people experience mild symptoms, others suffer debilitating symptoms that greatly impair their quality of life. Virtually all organs may be involved. The disease commonly affects the eyes, mouth, parotid gland, lungs, kidneys, skin, and nervous system. Sjogren's syndrome can also cause problems in other parts of the body, including the joints, lungs, muscles, kidneys, nerves, thyroid gland, liver, pancreas, stomach and brain.

Sjögren's occurs in all age groups in both women and men. It is estimated to strike as many as 4 million people in the United States. Sjogren's Syndrome is also found more commonly in families that have members with other autoimmune illnesses, such as systemic lupus erythematosus, autoimmune thyroid disease, juvenile diabetes, etc. Sjögren's syndrome—dryness, fatigue, pain, head and neck complaints, hoarseness, or hearing loss—can also occur as a result of medication use, anxiety and depression, or normal aging.  The majority of the patients with Sjögren’s syndrome have symptoms related to diminished lacrimal and salivary gland functions. The initial manifestations may be nonspecific, such as arthralgias, Raynaud’s phenomenon and fatigue, and usually 8 to 10 years elapse from the initial symptoms to the full-blown development of the disease. Patients usually complain of difficulty in eating dry food (like trying to eat crackers without water), inability to speak continuously, oral soreness, changes in tasting and smelling and fissures of the tongue and lips (angular queilitis).

Treatment is generally symptomatic and supportive. Infections are treated with anti-fungal or antibiotic preparations. Hydroxychloroquine (Plaquenil) has been helpful for some manifestations of Sjogren's syndrome. Moisture replacement therapies may ease the symptoms of dryness. Nonsteroidal anti-inflammatory drugs may be used to treat musculoskeletal symptoms. Lacriserts, is used by some to alleviate dry eyes. Two prescription medications, Salagen (pilocarpine hydrochloride) and Evoxac (cevimeline), are available to treat dry mouth.. Serious complications, such as vasculitis, can require immune suppression medications, including cortisone (prednisone and others) and/or azathioprine (Imuran) or cyclophosphamide (Cytoxan).  Many of these types of agents are available as over-the-counter products including toothpaste, gum, and mouthwash (Biotene). Vitamin E oil has been used with some success. Infections of the mouth and teeth should be addressed as early as possible in order to avoid more severe complications.