Sjögren’s syndrome is a chronic autoimmune disorder that primarily affects the glands producing tears and saliva, leading to symptoms of dryness in the eyes and mouth, but also can affect multiple other tissues.
The disorder is classified as either primary or secondary, depending on whether it appears as an isolated illness or follows another autoimmune disease, such as rheumatoid arthritis or systemic lupus erythematosus.
Causes of Sjögren’s syndrome
The immune system normally defends the body against “foreign” microorganisms that might cause disease, such as viruses and bacteria, as well as malfunctioning cells.
Sjögren’s syndrome is an autoimmune disease in which the immune system erroneously recognizes the body’s own cells as a foreign threat, and launches an inflammatory attack against those cells.
The disorder’s exact cause remains unknown. Scientists believe that a combination of genetics and an infection causes the immune system to start mistakenly attacking the body’s own cells.
What are the symptoms?
The misguided immune response in Sjögren’s syndrome patients is primarily targeted at the glands producing saliva and tears. As a result, dry eyes and mouth are the hallmark symptoms of Sjögren’s syndrome.
Other parts of the body also can be damaged, leading to a variety of symptoms. These include joint pain, swollen salivary glands, skin rashes, dry skin, vaginal dryness, a persistent dry cough, prolonged fatigue, and chronic pain.
Neurological problems, such as damage to the nerves outside the brain and spinal cord (peripheral neuropathy), cognitive disorders, or inflammation of the brain membranes (meningitis) are common in Sjögren’s. More rarely, patients may develop brain lesions.
Diagnosing Sjögren’s disease
Many symptoms of Sjögren’s mimic those of other disorders, and not all appear at the same time. Because of this the disease currently lacks a definitive diagnostic test and doctors generally will base a diagnosis on a combination of tests.
These include blood and urine tests to detect antibodies or markers of organ damage, measuring tear production, imaging glands for physical signs of damage, evaluating tissue samples (biopsies) for signs of inflammation, and collecting a detailed medical history from each patient.
Because of its unknown origin, treatment focuses on lessening symptoms and preventing the long-term complications they might cause. These complications may include infection and dental disease because of the protective role that saliva plays in washing away infectious agents and preventing dental decay.
Sjögren’s syndrome patients also appear to have a higher risk for lymphomas, a blood cancer that arises in lymph nodes. For this reason, regular medical checkups often are recommended.
Despite the risks of serious complications, most people living with Sjögren’s syndrome remain relatively healthy, experiencing milder forms of the illness.
The exact prevalence of Sjögren’s syndrome is unknown, owing largely to varying classification criteria and its diagnostic challenge. No clear and widely-accepted screening tool exists, for example, to determine which patients showing dry eyes should be referred for a Sjögren’s syndrome workup.
Nonetheless, current studies estimate that the syndrome occurs in about 0.1% to 4% of the general population.
Last updated: May 6, 2021
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