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Causes and symptoms
Sjögren's syndrome(shō′grənz, shœ′-) also
Sjögren's syndromeAn immunological disorder causing reduced secretion of many exocrine glands as a result of damage by antibodies produced by the body (autoantibodies). Affected glands are infiltrated with lymphocytes. Their malfunction causes severe dryness of the eyes, mouth and vagina. The syndrome is commonly associated with other immunological disorders, such as RHEUMATOID ARTHRITIS, SYSTEMIC LUPUS ERYTHEMATOSUS and MYASTHENIA GRAVIS. Treatment is by anti-inflammatory and cytotoxic drugs and symptomatic relief can be obtained from artificial tears, moistening mouth sprays and vaginal lubricants. (Henrik Samuel Conrad Sjögren, b. 1899, Swedish ophthalmologist).
|Mean LOS:||5.2 days|
|Description:||MEDICAL: Connective Tissue Disorders With CC|
Sjögren’s syndrome (SS) is the most common autoimmune rheumatic disorder after rheumatoid arthritis (RA). It is a chronic, progressive disease associated with other diseases, such as RA, in approximately 50% of the cases.
SS is characterized by failure of exocrine glands and by diminished tearing and salivary secretion (sicca complex). It results from chronic exocrine gland dysfunction, although the disorder may also involve other organs such as the lung and kidney. SS may be a primary disorder or it may be associated with connective tissue disorders, such as RA, scleroderma, systemic lupus erythematosus, and primary biliary cirrhosis. Tissue damage results either from infiltration by lymphocytes or from the deposition of immune complexes. The overall prognosis for patients with SS is good, and the disease seldom leads to significant complications.
The direct cause of SS is unknown. It seems likely that both environmental and genetic factors (see Genetic Considerations) contribute to its development. In a genetically susceptible individual, either bacterial or viral infection or exposure to pollen may be the catalyst for SS.
SS is believed to be a complex disorder with a genetic susceptibility and an environmental trigger. Associations with the human leukocyte antigen variants DRB1, DRB1∗03, and DQB1∗02 have been described.
Gender, ethnic/racial, and life span considerations
SS occurs mainly in women: 9 out of 10 patients are female. The mean age of occurrence is 50, although it has been reported in children who develop parotid gland enlargement. A small percentage of women who develop SS may have accompanying nonlymphoma and lymphoid malignancies. No racial and ethnic considerations are known.
Global health considerations
SS occurs around the globe with similar prevalence as found in North America.
Establish a history of either autoimmune or lymphoproliferative disorders. Rule out other causes of oral and ocular dryness; ask about any history of sarcoidosis, endocrine disorders, anxiety or depression, and radiation therapy to the head and neck. Many commonly used medications produce dry mouth as a side effect, so take a thorough history of medications. In patients with salivary gland enlargement and severe lymphoid infiltration, rule out malignancy. Approximately 50% of patients with SS have confirmed RA. When you ask about symptoms, the patient may report gritty or sandy sensations in the eye or a film across the visual field. Patients may also report dryness of the mouth, burning oral discomfort, difficulty in chewing and swallowing dry foods, increased thirst, and reduced taste. The patient may also report the incidence of many dental caries and chronic middle ear infections. Dryness of the vagina and vulva leads to reports of painful urination, itching, and painful or difficult sexual intercourse.
The most common symptoms are dry eyes and dry mouth. The patient’s tongue is often red and dry with atrophic taste buds. Unilateral or bilateral parotid and salivary glands may be hardened and nontender. Dental caries are a common finding. The dryness may make talking difficult. Patients may have a dry, chronic cough and an increased incidence of upper and lower respiratory tract infections, which has resulted in a chronic vocal hoarseness. Nasal mucosa may be dry and reddened. Gastrointestinal tract involvement may lead to gastritis, esophageal mucosal atrophy, and difficulty in swallowing. Genitalia may appear dry and possibly ulcerated. Involvement of the exocrine glands leads to dry, tough, scaly skin; decreased sweat; and chronic itching.
The patient with SS has complaints that may have been attributed to multiple causes, possibly over years. Because SS is closely related to systemic lupus erythematosus and RA, the patient may have been misdiagnosed, causing considerable emotional distress. Because SS affects senses, such as sight and taste, and also sexuality, assess the patient’s ability to cope with the presenting symptoms and other common complaints.
|Test||Normal Result||Abnormality With Condition||Explanation|
|Salivary gland biopsy||Normal salivary gland cells||Presence of inflammatory cells and immune complexes||Identifies abnormal cells in secretory glands and ducts|
|Slit-lamp examination||Normal examination||Detection of dryness of conjunctiva and reduced tearing||Identifies reduced tear film and dryness of eyes|
Other Tests: Complete blood count, antinuclear antibodies, rheumatoid factor, anti-alpha-fodrin antibody, erythrocyte sedimentation rate, tear osmolarity, fluorescence clearance test, parotid flow rate, radionuclide scan, Schirmer test (test strip of number 41 Whatman filter paper placed near the lower conjunctival sac to measure tear formation; healthy persons wet 15 mm or more after 5 minutes)
Primary nursing diagnosis
DiagnosisImpaired skin integrity related to diminished or absent glandular secretions
OutcomesTissue integrity: Skin and mucous membranes; Wound healing: Primary intention; Knowledge: Treatment regimen; Self-care: Hygiene; Treatment behavior: Illness
InterventionsSkin and membrane surveillance; Skin care; Medication administration; Fluid balance; Surveillance
Planning and implementation
Care of the patient with SS is designed to treat symptoms. Instill artificial tears as often as every 30 minutes to prevent corneal ulcerations or opacifications that may be caused by insufficient lacrimal secretions.
Patients who also have RA may benefit from a combined program of medical, rehabilitative, and surgical treatments. The primary goals are suppression of further joint and tissue inflammation, maintenance of joint and tissue function, repair of joint damage, and relief of pain.
|Medication or Drug Class||Dosage||Description||Rationale|
|Cyclosporine ophthalmic eye drops||Apply 1% or 2% solution bid||Immunosuppressant||Suppresses T-cell formation and has been used to increase lacrimal gland function in SS|
Other Drugs: Corticosteroids; eye lubricants; omega-3 fish oil; sustained-release cellulose capsules (hydroxypropyl cellulose) may be used. Topical (diquafosol) and systematic (pilocarpine, cevimeline) stimulators of tear secretion may be used. If eye infection develops, the patient receives antibiotics; topical steroids are avoided.
Suggest the use of sunglasses to protect the patient’s eyes from strong light, wind, and dust. To reduce the risk of infection caused by dry eyes, advise the patient to keep his or her face clean and to avoid rubbing the eyes. Mouth dryness can be relieved by using a swab or spray and by drinking plenty of fluids, especially at mealtime. Sugarless throat lozenges can also relieve mouth dryness without promoting tooth decay. Meticulous oral hygiene should include regular brushing, flossing, and fluoride treatment at home, along with frequent dental checkups. Teach the patient to avoid medications that decrease saliva production, such as atropine derivatives, antihistamines, anticholinergics, and antidepressants. Suggest high-protein, high-calorie liquid supplements to patients with painful mouth lesions. Soft foods may be easier for patients to swallow. Parotid gland enlargement can be treated with local heat and analgesia.
Respiratory dryness can be reduced by using a humidifier at home and at work. Nasal dryness can be relieved by the use of normal saline solution drops. Moisturizing lotions can ease skin dryness, as can avoiding lengthy hot showers or baths. Patients should avoid sunburn and any lengthy exposure to the sun; recommend using a sunscreen when outdoors. Water-soluble lubricating jelly is an effective lubricant during sexual intercourse.
Evidence-Based Practice and Health Policy
Segal, B.M., Pogatchnik, B., Holker, E., Liu, H., Sloan, J., Rhodus, N., & Moser, K.L. (2013). Primary Sjogren’s syndrome: Cognitive symptoms, mood, and cognitive performance. Acta Neurologica Scandinavica, 125(4), 272–278.
- In a study comparing 39 adult patients with primary SS to 17 healthy control participants, 47% of SS patients reported depression compared to 6% of control participants.
- Depression was significantly correlated with cognitive functioning (r, 0.782; p < 0.001). Compared to control participants, patients with SS performed worse in psychomotor processing (p = 0.027) and verbal reasoning (p = 0.007).
- Physical findings of dysphagia (difficulty swallowing)
- Physical findings of presence of red, irritated, or ulcerated mucosal membranes
- Reaction to remoisturizing eyes, mouth, and other affected areas
Discharge and home healthcare guidelines
Instruct the patient to avoid sugar; tobacco; alcohol; and spicy, salty, and highly acidic foods. Recommend high-calorie, protein-rich liquid supplements to patients with painful mouth lesions. Teach the patient how to instill eye drops, ointments, or sustained-release capsules. Advise the patient to avoid over-the-counter medications that include saliva-decreasing compounds, such as antihistamines, antidepressants, anticholinergics, and atropine derivatives.