23/07/2022
What Is Sjögren's Disease?
Sjögren's (SHOW’-grenz) is a systemic autoimmune, rheumatic disease that affects the entire body. The most common problems are dry mouth, dry eyes, fatigue and musculoskeletal pain. However, other potential complications include other areas of dryness, internal organ involvement, neurological complications, and lymphomas.
Ten times as many women as men are diagnosed with Sjögren's. While most often diagnosed in women during middle age or after menopause, Sjögren's can affect people of any age, including children. Younger Sjögren's patients often present differently compared to adults.
Sjögren's patients should be monitored closely for risk factors and development of blood cancers, especially non-Hodgkin B cell lymphoma (see below).
Pregnant Sjögren's patients who are positive for the autoantibody SSA/Ro should seek care from a perinatologist (obstetrician who manages high risk pregnancies) and be monitored for signs of fetal heart block.
About half of those with Sjögren's have another autoimmune disease, such as rheumatoid arthritis, lupus, or scleroderma, so patients with other autoimmune diseases should also be assessed for signs of Sjögren's.
No systemic immunomodulating therapy has been FDA-approved for Sjögren's. However, many potential treatments are used and others being investigated. Patients may be treated with nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, hydroxychloroquine, and other drugs that calm the immune system. Treatments for dryness aim to relieve symptoms and prevent complications such as thrush, other infections, and dental damage.
According to the 2008 National Arthritis Data Workgroup report (which included the American College of Rheumatology) on the prevalence of rheumatic diseases, Sjögren's patients without an accompanying, major autoimmune connective tissue disease number between 400, 000 and 3.1 million adults and, when including patients who also have another related disease, the prevalence is twice that number. In treating Sjögren's patients, embracing a multidisciplinary team approach and communication with other medical disciplines is critical for optimal management.
In the 1930s, Swedish physician Henrik Sjögren described a group of women who had profound dry eyes and dry mouth many of whom also had chronic arthritis. Today, rheumatologists know more about the disease named for Sjögren and can offer patients advice about how to live and manage it.
What Is Sjögren's Disease?
Sjögren's is a systemic, autoimmune, rheumatic disease that can affect many different body parts, especially the moisture-producing glands, and cause widespread dryness and other serious problems. Although dry mouth and dry eyes are the most common symptoms, dryness can also occur in the nose, sinuses, ears, throat, skin, and, in women, the va**na. There is speculation that, in men with Sjögren's, the prostate might be affected similarly to other organs. These problems may interfere with work, social activities, and quality of life. Patients may notice irritation, a gritty feeling, or painful burning in the eyes; dry eyes are at increased risk for infection and susceptible to corneal damage if not treated. Dry mouth can lead to difficulty eating and swallowing dry foods. It can lead to dental cavities, chipping, breaking, and loss of teeth. Dry mouth may increase gingivitis (gum inflammation) and oral yeast infections (candida) that may cause pain and burning. Sjögren's frequently causes swelling of the parotid glands (the glands below the ears and run along the jawline). Dryness can increase infections in the eyes, mouth, sinuses, lungs, and va**na.
Sjögren's can also affect the joints, muscles, nervous system (central nervous system and peripheral nervous system, including the autonomic nervous system), gastrointestinal tract (including the pancreas and liver), skin, blood vessels, lungs, and kidneys. Joint pain and stiffness with mild swelling are common, even in those without rheumatoid arthritis. Rashes may occur, including inflammation of small blood vessels (vasculitis), most commonly on the lower legs. Sun-sensitive rash is more common on the back, chest, face, and arms. Peripheral neuropathy can cause numbness and tingling, especially in the feet, and can frequently pre-date symptoms of dryness.Fatigue, cognitive dysfunction, and sleep abnormalities are frequently reported symptoms.Patients should also be monitored for depression and anxiety. .
Sjögren's can be accompanied by other autoimmune connective tissue disorders, such as systemic lupus erythematosus, rheumatoid arthritis, scleroderma, autoimmune thyroid disease, antiphospholipid syndrome, sarcoidosis, and celiac disease. It can occasionally be confused with fibromyalgia, multiple sclerosis
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