| Sjögren's syndrome is a chronic autoimmune disease in which white blood cells (lymphocytes) invade fluid-secreting glands, such as the tear (lacrimal) glands and the salivary glands.
The invading lymphocytes destroy gland tissue, leading to gland dysfunction. Although the lacrimal and salivary glands are the most commonly affected, Sjögren's syndrome is a whole body (systemic) disease that can affect any organ. As a result, diagnosis is often delayed, and many cases of Sjögren's syndrome may not be diagnosed.
Sjögren's syndrome is classified into primary and secondary disease. In primary disease the syndrome occurs in the absence of any other autoimmune disorder. Secondary disease refers to Sjögren's syndrome in individuals who have another autoimmune disease, such as scleroderma, polymyositis, lupus, or rheumatoid arthritis.Risk: The cause of Sjögren's syndrome is unknown, although viral infection, sex hormones, and a genetic predisposition have been implicated as triggers for the autoimmune process. It is estimated that this disease affects 1 million to 4 million individuals in the US (Mayo Clinic Staff). Ninety percent of individuals with Sjögren's syndrome are women. The syndrome is typically diagnosed in individuals over age 40. Incidence and Prevalence: Because there is no broadly accepted set of diagnostic criteria for Sjögren's syndrome, and because the disease often causes variable and nonspecific symptoms in some individuals, the incidence and prevalence are unknown. |
Source: Medical Disability Advisor
| History: The individual may complain of dry eyes (xerophthalmia) that feel as if they contain grit or sand and dry mouth (xerostomia). Individuals may report that swallowing is difficult (pharyngeal dysphagia) and that they must drink liquids when eating. Fifty percent of individuals with Sjögren's syndrome report fatigue and symptoms of fibromyalgia. Rarely, individuals with Sjögren's syndrome present with other symptoms, including joint pain (arthralgia), diarrhea, muscle weakness, headaches, dry cough, or multiple sclerosis-like symptoms. Physical exam: The individual may have red, irritated eyes and may blink or rub the eyes excessively. Examination of the mouth and teeth may reveal inadequate saliva production and possibly tooth decay (dental caries). The parotid glands may be enlarged. Exposure of the hands to cold temperatures may cause extreme whitening of the fingers (Raynaud's phenomenon). Tests: Blood tests may include erythrocyte sedimentation rate (ESR), complete blood count (CBC), antinuclear antibodies (ANA), rheumatoid factor, and SS-A and SS-B antibodies. Schirmer's test, in which absorbent paper strips are inserted into the lower eyelid, may be performed to quantify tear production. Rose bengal dye may be placed in the eye to evaluate the cornea. Biopsy of the parotid gland or other salivary glands may be conducted to detect the presence of inflammatory cells. A special x-ray (sialogram) may be used to evaluate the flow of saliva into the mouth. A urine sample may to taken to determine if the disease has affected the kidneys. |
Source: Medical Disability Advisor
| There is no cure for Sjögren's syndrome, so treatment is aimed at relieving symptoms and preventing or managing complications. Dry eyes are treated with lubricating eyedrops, moisture chamber panels, contact lenses, and/or glasses. Pilocarpine may be used to stimulate tear or saliva production. The individual should avoid drying factors (e.g., hair dryers, medications such as antihistamines and decongestants that cause drying, wind). Blockage of the lower tear ducts (punctal occlusion) with silicone plugs can conserve natural tears. Dry mouth is treated with saliva substitutes or sugar-free lemon hard candy (to stimulate salivation); the prescription medicine cevimeline is sometimes used. The individual needs to be meticulous regarding oral hygiene, use mechanical cleaning devices, have frequent dental check ups, and receive fluoride treatments. Humidifiers should be used at work and at home. Analgesics and NSAIDs are used to treat arthralgia or other pains. Oral corticosteroids may be given to reduce inflammation and slow the damage to joints. Sometimes an antimalarial drug is given to individuals with inflamed joints. |
Source: Medical Disability Advisor
| Sjögren's syndrome is a chronic, systemic disease for which no cure exists. It can have a substantial impact on quality of life. Strict adherence to treatment guidelines and frequent evaluation by a physician can minimize symptoms and lead to a favorable outcome. |
Source: Medical Disability Advisor
| Tooth decay, chronic oral yeast infection (candidiasis), wasting (atrophy) of the oral mucosa, atrophy of the tongue, small cracks (fissures) on the tongue, irritation and destruction of the membrane that lines the eyelids and exposed surface of the eyeball (conjunctiva), hearing loss, kidney (renal) disease, pneumonia, and skin lesions are potential complications. Individuals with Sjögren's syndrome are at a greater risk of developing malignant lymphoma. |
Source: Medical Disability Advisor
| Individuals should avoid dry surroundings and may need a humidifier for the office. Individuals who work outside may need to be reassigned to indoor duties in order to avoid wind. |
Source: Medical Disability Advisor
| If an individual fails to recover within the expected maximum duration period, the reader may wish to consider the following questions to better understand the specifics of an individual's medical case. Regarding diagnosis:
- Did individual present with characteristic symptoms of dry eyes, dry mouth, difficulty in swallowing, fatigue, or joint and muscle pain?
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Were diagnostic findings consistent with a diagnosis of Sjögren's syndrome (i.e., presence of rheumatoid factor, elevated ANA titers, presence of SS-A and SS-B antibodies, etc)?
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If the diagnosis was uncertain, were other conditions with similar symptoms (such as mumps, sarcoidosis, parotid tumors) ruled out?
Regarding treatment:
- Has treatment successfully relieved the symptoms?
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Has individual been instructed regarding self-care techniques to help reduce symptoms (such as hard candies to stimulate saliva, meticulous oral hygiene, use of humidifiers)?
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Is individual complying with the treatment recommendations?
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Would additional counseling and instruction be beneficial?
Regarding prognosis:
- Is individual compliant with treatment plan? What can be done to enhance compliance?
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Have appropriate work accommodations been made to minimize individual's symptoms (humidifiers)?
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Has individual suffered any associated complications (such as tooth decay, oral infections, eye inflammation) that could impact recovery and prognosis?
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Does individual have any comorbid conditions, such as rheumatoid arthritis, scleroderma, HIV, or cancer, that may impact recovery and prognosis?
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Source: Medical Disability Advisor
| Mayo Clinic Staff. "Sjogren's Syndrome." MayoClinic.com. 26 Nov. 2003. Mayo Foundation for Medical Education and Research. 29 Dec. 2004 <http:www.mayoclinic.com/invoke.cfm?id=DS00147>. |
Source: Medical Disability Advisor
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