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    Diagnosing and treating Sjögren's syndrome

    Sjögren’s syndrome is a chronic inflammatory disorder that affects 0.1 percent to 0.6 percent of the population and is estimated to affect as many as four million people in the United States. This is roughly equivalent to the same number of patients with rheumatoid arthritis. However, only just over one million individuals have been diagnosed with Sjögren’s syndrome.1

    Sjogren's patient with punctate epithelial keratopathy.This gap alludes to the difficulty in identifying the disease because it has an incipient course and initially presents with non-specific symptoms. Many of the symptoms present early in the disease may be confused with other matters common to such items as menopause, medication side effects, and non-specific aging changes. As a result, the time between initiation of symptoms associated with the disease and a confirmatory diagnosis is often 3.5 years or longer.1

    Related: Understanding Sjögren's syndrome

    Sjögren’s syndrome may also present as a primary disease, meaning it occurs alone, or as a secondary disorder, occurring along with other chronic inflammatory disorders—most commonly rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), scleroderma, or polymyositis.2 The disease is found in both sexes, affects females roughly nine times more than males, and occurs in virtually any age range.  The “typical” patient is female, Caucasian, and of middle to older age.3

    Sjögren pathophysiology

    The key factor of the disease is exocrine gland dysfunction where the body’s immune system begins to attack several key secretory glands, including the lacrimal and salivary glands. As such, two of the primary clinical features are keratoconjunctivitis sicca and xerostomia, which are hallmark of the clinical diagnosis. 

    Related: Identify Sjögren's patients with Sjö test

    However, this is only the tip of the iceberg. The disease often also affects other organ systems, including the nervous system, skin, lungs, and kidneys. In primary Sjögren’s syndrome, up to 75 percent of patients manifest with extraglandular disease.4 As such, the list of other symptoms associated with Sjögren’s syndrome involves dental decay, debilitating fatigue, dry skin, peripheral neuropathy, arthropathy, and gastrointestinal problems.

    The cumulative risk of a Sjögren’s patient to develop non-Hodgkin’s B-cell lymphoma within 15 years of her diagnosis is 9.8 percent—significantly higher than that of the general population.5 In addition, 20 to 30 percent of patients with primary Sjögren’s syndrome have clinical pulmonary involvement, which is associated with lower quality of life and increased 10-year mortality.6


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