Sjögren's syndrome
Sjögren's syndrome: Excerpt from Professional Guide to Diseases (Eighth Edition)
The second most common autoimmune rheumatic disorder after rheumatoid arthritis (RA), Sjögren’s syndrome is characterized by diminished lacrimal and salivary gland secretion (sicca complex). Sjögren’s syndrome may be a primary disorder or it may be associated with connective tissue disorders, such as RA, scleroderma, systemic lupus erythematosus, and polymyositis. In some patients, the disorder is limited to the exocrine glands (glandular Sjögren’s syndrome); in others, it also involves other organs, such as the lungs and kidneys (extraglandular Sjögren’s syndrome).
Causes and incidence
The cause of Sjögren’s syndrome is unknown, but genetic and environmental factors probably contribute to its development. Viral or bacterial infection or perhaps exposure to pollen may trigger Sjögren’s syndrome in a genetically susceptible individual. Tissue damage results from infiltration by lymphocytes or from the deposition of immune complexes. Lymphocytic infiltration may be classified as benign, malignant, or pseudolymphoma (nonmalignant, but tumorlike aggregates of lymphoid cells).
This syndrome occurs mainly in females (90% of patients); mean age of onset is 40 to 50.
Signs and symptoms
About 50% of patients with Sjögren’s syndrome have confirmed RA and a history of slowly developing sicca complex. However, some patients seek medical help for rapidly progressive and severe oral and ocular dryness, in many cases accompanied by periodic parotid gland enlargement. Ocular dryness (xerophthalmia) leads to foreign body sensation (gritty, sandy eye), redness, burning, photosensitivity, eye fatigue, itching, and mucoid discharge. The patient may also complain of a film across his field of vision.
Oral dryness (xerostomia) leads to difficulty swallowing and talking; abnormal taste or smell sensation or both; thirst; ulcers of the tongue, buccal mucosa, and lips (especially at the corners of the mouth); and severe dental caries. Dryness of the respiratory tract leads to epistaxis, hoarseness, chronic nonproductive cough, recurrent otitis media, and increased incidence of respiratory infections.
Other effects may include dyspareunia and pruritus (associated with vaginal dryness), generalized itching, fatigue, recurrent low-grade fever, and arthralgia or myalgia. Lymph node enlargement may be the first sign of malignant lymphoma or pseudolymphoma.
Specific extraglandular findings in Sjögren’s syndrome include interstitial pneumonitis; interstitial nephritis, which results in renal tubular acidosis in 25% of patients; Raynaud’s phenomenon (20%); and vasculitis, usually limited to the skin and characterized by palpable purpura on the legs (20%). About 50% of patients show evidence of hypothyroidism related to autoimmune thyroid disease. A few patients develop systemic necrotizing vasculitis.
Diagnosis
Diagnosis of Sjögren’s syndrome rests on the detection of two of the following three conditions: xerophthalmia, xerostomia (with salivary gland biopsy showing lymphocytic infiltration), and an associated autoimmune or lymphoproliferative disorder. Diagnosis must rule out other causes of oral and ocular dryness, including sarcoidosis, endocrine disorders, anxiety or depression, and effects of therapy such as radiation to the head and neck. More than 200 commonly used drugs also produce dry mouth as an adverse effect. In patients with salivary gland enlargement and severe lymphoid infiltration, diagnosis must rule out cancer.
Laboratory values include elevated erythrocyte sedimentation rate in most patients, mild anemia and leukopenia in 30%, and hypergammaglobulinemia in 50%. Autoantibodies are also common, including anti-Sjögren’s syndrome-A (anti-Ro) and anti-Sjögren’s syndrome-B (anti-La), which are antinuclear and antisalivary duct antibodies. From 75% to 90% of patients test positive for rheumatoid factor; 90%, for antinuclear antibodies.
Other tests help support this diagnosis. Schirmer’s tearing test and slit-lamp examination with rose bengal dye are used to measure eye involvement. Salivary gland involvement is evaluated by measuring the volume of parotid saliva and by secretory sialography and salivary scintigraphy. Lower-lip biopsy shows salivary gland infiltration by lymphocytes.
Treatment
Treatment is usually symptomatic and includes conservative measures to relieve ocular or oral dryness. Mouth dryness can be relieved by using a methylcellulose swab or spray and by drinking plenty of fluids, especially at mealtime. Meticulous oral hygiene is essential, including regular flossing, brushing, at-home fluoride treatment, and frequent dental checkups.
Instill artificial tears as often as every half hour to prevent eye damage (corneal ulcerations and corneal opacifications) from insufficient tear secretions. Some patients may also benefit from instillation of an eye ointment at bedtime or from twice-a-day use of sustained-release cellulose capsules (Lacrisert). If infection develops, antibiotics should be given immediately; topical steroids should be avoided. Other treatment measures vary with associated extraglandular findings. Parotid gland enlargement requires local heat and analgesics. Pulmonary and renal interstitial disease necessitate corticosteroid use. Accompanying lymphoma is treated with a combination of chemotherapy, surgery, or radiation.
Special considerations
❑ Stress the need to humidify home and work environments to help relieve respiratory dryness.
❑ Advise the patient to avoid drugs that decrease saliva production, such as atropine derivatives, antihistamines, anticholinergics, and antidepressants.
❑ If mouth lesions make eating painful, suggest high-protein, high-calorie liquid supplements to prevent malnutrition.
❑ Advise the patient to avoid sugar, which contributes to dental caries. Tobacco; alcohol; and spicy, salty, or highly acidic foods, which cause mouth irritation, should also be avoided.
❑ Suggest normal saline solution drops or aerosolized spray for nasal dryness.
❑ Advise the patient to avoid prolonged hot showers and baths and to use moisturizing lotions to help ease dry skin. Suggest K-Y lubricating jelly as a vaginal lubricant.
❑ Suggest the use of sunglasses to protect the patient’s eyes from dust, wind, and strong light. Moisture chamber spectacles may also be helpful. Because dry eyes are more susceptible to infection, advise the patient to keep his face clean and to avoid rubbing his eyes.
❑ Refer the patient to the Sjögren’s Syndrome Foundation for additional information and support.
Book Source Details
- Book Title: Professional Guide to Diseases (Eighth Edition)
- Author(s): Springhouse
- Year of Publication: 2005
- Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright © 2005 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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