Sjögren's syndrome

Sjögren's Syndrome

 

Definition

Sjögren's syndrome (SS) is a disorder in which the mouth and eyes become extremely dry. Sjögren's syndrome is often associated with other autoimmune disorders. It is named for Henrik Sjögren, a Swedish ophthalmologist.

Description

Like other autoimmune disorders, Sjögren's syndrome occurs when the body's immune system mistakenly begins treating parts of the body as foreign invaders. While the immune cells should attack and kill invaders like bacteria, viruses, and fungi, these cells should not attack the body itself. In autoimmune disorders, however, cells called antibodies see tissues of the body as foreign, and help to start a chain of events that results in damage and destruction of those tissues.

There are three types of Sjögren's syndrome. Primary Sjögren's syndrome occurs by itself, with no other associated disorders. Secondary Sjögren's syndrome occurs along with other autoimmune disorders, like systemic lupus erythematosus, rheumatoid arthritis, scleroderma, vasculitis, or polymyositis. When the disorder is limited to involvement of the eyes, with no other organ or tissue involvement evident, it is called sicca complex.

Women are about nine times more likely to suffer from Sjögren's syndrome than are men. SS affects all age groups, although most patients are diagnosed when they are between 40 and 55 years old. Sjögren's syndrome is commonly associated with other autoimmune disorders. In fact, 30% of patients with certain autoimmune disorders will also have Sjögren's syndrome.

SS is found in all races and ethnic groups. It is thought to affect between 0.1% and 3% of the population in the United States; this range reflects the lack of a uniform set of diagnostic criteria. According to the American College of Rheumatology, between 1 million and 4 million Americans have Sjögren's syndrome.

Causes and symptoms

The cause of Sjögren's syndrome has not been clearly defined, but several causes are suspected. The syndrome sometimes runs in families. Other potential causes include hormonal factors (since there are more women than men with the disease) and viral factors. The viral theory suggests that the immune system is activated in response to a viral invader, but then fails to turn itself off. Some other immune malfunction then causes the overly active immune system to begin attacking the body's own tissues. In 2004 a group of Greek researchers presented evidence that a coxsackievirus may be the disease organism that triggers SS.

The main problem in Sjögren's syndrome is dryness. The salivary glands are often attacked and slowly destroyed, leaving the mouth extremely dry and sticky. Swallowing and talking become difficult. Normally, the saliva washes the teeth clean. Saliva cannot perform this function in Sjögren's syndrome, so the teeth develop many cavities and decay quickly. The parotid glands produce the majority of the mouth's saliva. They are located lying over the jaw bones behind the area of the cheeks and in front of the ears, and may become significantly enlarged in Sjögren's syndrome.

The eyes also become extremely dry as the tear glands (called glands of lacrimation) are slowly destroyed. Eye symptoms include itching, burning, redness, increased sensitivity to light, and thick secretions gathering at the eye corners closest to the nose. The cornea may have small irritated pits in its surface (ulcerations).

Destruction of glands in other areas of the body may cause a variety of symptoms. In the nose, dryness may result in nosebleeds. In the rest of the respiratory tract, the rates of ear infection, hoarseness, bronchitis, and pneumonia may increase. Vaginal dryness can be quite uncomfortable. Rarely, the pancreas may slow production of enzymes important for digestion. The kidney may malfunction. About 33% of all patients with Sjögren's syndrome have other symptoms unrelated to gland destruction. These symptoms include fatigue, decreased energy, fevers, muscle aches and pains, and joint pain.

Many patients with SS also develop a variety of skin problems that include dry patches, vasculitis, and cutaneous B-cell lymphoma. These and other dermatologic disorders are more common in SS than was previously thought.

Patients who also have other autoimmune diseases will suffer from the symptoms specific to those conditions.

In addition to physical symptoms, patients with SS appear to be at increased risk for depression and other mood disorders.

Diagnosis

Diagnosis of Sjögren's syndrome is based on the patient having at least three consecutive months of bothersome eye and/or mouth dryness. A variety of tests can then be done to determine the quantity of tears produced, the quantity of saliva produced, and the presence or absence of antibodies that could be involved in the destruction of glands.

Treatment

There is no cure for Sjögren's syndrome. Instead, treatment usually attempts to reduce the discomfort and complications associated with dryness of the eyes and mouth (and other areas). Artificial tears are available, and may need to be used up to every 30 minutes. By using these types of products, the patient is more comfortable and avoids the complications associated with eyes that are overly dry. Dry mouth is treated by sipping fluids slowly but constantly throughout the day. Sugarless chewing gum can also be helpful. An artificial saliva is available for use as a mouthwash. Patients may also be given such drugs as pilocarpine (Salagen) or cevimeline (Evoxac) to increase saliva and tear secretions. Careful dental hygiene is important in order to avoid tooth decay, and it is wise for patients to decrease sugar intake. Vaginal dryness can be treated with certain gel preparations. Steroid medications may be required when other symptoms of autoimmune disorders complicate Sjögren's syndrome. However, these medications should be avoided when possible because they may make the cornea thin and even more susceptible to injury.

Key terms

Autoimmune disorder — A disorder in which the body's immune cells mistake the body's own tissues as foreign invaders; the immune cells then work to destroy tissues in the body.

Cornea — A transparent structure of the eye over the iris and pupil; light must pass through the cornea to make vision possible.

Coxsackievirus — Any of a group of enteroviruses that produce a disease in humans characterized by fever and rash. Coxsackieviruses are named for the town in upstate New York where they were first identified.

Immune system — The complex network of organs and blood cells that protect the body from foreign invaders, like bacteria, viruses, and fungi.

Prognosis

The prognosis for patients with primary Sjögren's syndrome is particularly good; these patients have a normal life expectancy. Although the condition is quite annoying, serious complications rarely occur. The prognosis for patients with secondary Sjögren's syndrome varies since it depends on the prognosis for the accompanying autoimmune disorder.

Prevention

Since the cause of Sjögren's syndrome is unknown as of 2004, there are no known ways to prevent this syndrome.

Resources

Books

Beers, Mark H., MD, and Robert Berkow, MD., editors. "Diffuse Connective Tissue Disease." Section 5, Chapter 50 In The Merck Manual of Diagnosis and Therapy. Whitehouse Station, NJ: Merck Research Laboratories, 2004.

Moutsopoulos, Haralampos M. "Sjögren's Syndrome." In Harrison's Principles of Internal Medicine, edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1997.

Periodicals

Bell, Mary, et al. "Sjögren's Syndrome: A Critical Review of Clinical Management." The Journal of Rheumatology 26, no. 9 (2001): 2051-2059.

Francis, Mark L., MD. "Sjogren Syndrome." eMedicine July 1, 2004. http://emedicine.com/med/topic2136.htm.

Ono, M., E. Takamura, K. Shinozaki, et al. "Therapeutic Effect of Cevimeline on Dry Eye in Patients with Sjögren's Syndrome: A Randomized, Double-Blind Clinical Study." American Journal of Ophthalmology 138 (July 2004): 6-17.

Roguedas, A. M., L. Misery, B. Sassolas, et al. "Cutaneous Manifestations of Primary Sjögren's Syndrome Are Underestimated." Clinical and Experimental Rheumatology 22 (September-October 2004): 632-636.

Stevenson, H. A., M. E. Jones, J. L. Rostron, et al. "UK Patients with Primary Sjögren's Syndrome Are at Increased Risk from Clinical Depression." Gerodontology 21 (September 2004): 141-145.

Triantafyllopoulou, A., N. Tapinos, and H. M. Moutsopoulos. "Evidence for Coxsackievirus Infection in Primary Sjögren's Syndrome." Arthritis and Rheumatism 50 (September 2004): 2897-2902.

Organizations

American College of Rheumatology. 1800 Century Place, Suite 250, Atlanta, GA 30345-4300. (404) 633-3777. Fax: (404) 633-1870. http://www.rheumatology.org.

Sjögren's Syndrome Foundation, Inc. 8120 Woodmont Avenue, Bethesda, MD 20814. (800) 475-6473. Fax: (301) 718-0322. http://www.sjogrens.org.

Other

American College of Rheumatology Fact Sheet. "Sjögren's Syndrome." http://www.rheumatology.org/public/factsheets/sjogrens_new.asp?aud=pat.

Sjögren's syndrome

 [sha´grenz]

a symptom complex of unknown etiology, usually occurring in middle-aged or older women, marked by the triad of keratoconjunctivitis sicca with or without lacrimal gland enlargement, xerostomia with or without salivary gland enlargement, and the presence of a connective tissue disease, usually rheumatoid arthritis but sometimes systemic lupus erythematosus, scleroderma, or polymyositis. An abnormal immune response has been implicated. The dryness and the discomfort related to the lack of secretions are treated symptomatically.

Sjögren's syndrome

(shō′grənz, shœ′-) also

Sjögren syndrome

(-grĕn)

n.

A chronic autoimmune disease occurring mostly in older women, characterized by keratoconjunctivitis, dryness of mucous membranes, joint pain, fatigue, and enlargement of the parotid glands. It is often associated with rheumatoid arthritis, Raynaud's phenomenon, and dental caries.

Sjögren's syndrome

An 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).

Sjögren’s Syndrome

DRG Category:	546
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.

Causes

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.

Genetic considerations

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.

Assessment

History

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.

Physical examination

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.

Psychosocial

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.

Diagnostic highlights

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

Diagnosis

Impaired skin integrity related to diminished or absent glandular secretions

Outcomes

Tissue integrity: Skin and mucous membranes; Wound healing: Primary intention; Knowledge: Treatment regimen; Self-care: Hygiene; Treatment behavior: Illness

Interventions

Skin and membrane surveillance; Skin care; Medication administration; Fluid balance; Surveillance

Planning and implementation

Collaborative

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.

Pharmacologic highlights

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.

Independent

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).

Documentation guidelines

• 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.