Sjögren's syndrome
Sjögren's syndrome: Excerpt from Handbook of Diseases
The second most common autoimmune rheumatic disorder after rheumatoid arthritis (RA), Sjögren’s syndrome (SS) is characterized by diminished lacrimal and salivary gland secretion (sicca complex). SS occurs mainly in women (90% of patients); its mean age of occurrence is 50.
SS may be a primary disorder or may be associated with a connective tissue disorder, such as RA, scleroderma, systemic lupus erythematosus, or polymyositis. In some patients, the disorder is limited to the exocrine glands (glandular SS); in others, it also involves other organs, such as the lungs and kidneys (extraglandular SS).
Causes
The cause of SS is unknown. Most likely, genetic and environmental factors contribute to its development. Viral or bacterial infection or perhaps exposure to pollen may trigger SS 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 lymphoma, malignant lymphoma, or pseudolymphoma (nonmalignant but tumorlike aggregates of lymphoid cells).
Signs and symptoms
About 50% of patients with SS have confirmed RA and a history of slowly developing sicca complex. However, some seek medical help for rapidly progressive and severe oral and ocular dryness, often accompanied by periodic parotid gland enlargement.
Ocular effects
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 effects
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 features
Other effects may include dyspareunia and pruritus (associated with vaginal dryness), generalized itching, fatigue, recurrent low-grade fever, and arthralgia or myalgia.
CLINICAL TIP: Lymphadenopathy is relatively common in patients with SS, but persistent, firm lymph node enlargement may be the first sign of malignant transformation. Patients with primary SS have a 44 times greater relative risk of lymphoma than does the general population.
Specific extraglandular findings in SS include interstitial pneumonitis; interstitial nephritis, which can result in renal tubular acidosis; Raynaud’s phenomenon; arthritis and arthralgias; peripheral neuropathy; and vasculitis, usually limited to the skin and characterized by palpable purpura on the legs.
About 50% of patients show evidence of hypothyroidism related to autoimmune thyroid disease. A few patients develop systemic necrotizing vasculitis.
Diagnosis
A patient with SS has at least two of the following conditions: xerophthalmia, xerostomia (with a salivary gland biopsy showing lymphocytic infiltration), and an associated autoimmune or lymphoproliferative disorder.
Tests to rule out other causes
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. Over 200 commonly used drugs also produce dry mouth.
In patients with salivary gland enlargement and severe lymphoid infiltration, the diagnosis must rule out cancer.
Laboratory tests
Patients with SS test positive for antinuclear antibodies. A salivary gland biopsy will also return a positive result. Laboratory values include an elevated erythrocyte sedimentation rate in most patients, mild anemia and leukopenia in 30%, and hypergammaglobulinemia in 50%; 75% to 90% of patients test positive for rheumatoid factor.
Other tests
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. A lower lip biopsy shows salivary gland infiltration by lymphocytes.
Treatment
Usually symptomatic, treatment includes conservative measures to relieve ocular or oral dryness.
Symptomatic treatment
Mouth dryness can be relieved by using a methylcellulose swab or spray and by drinking plenty of fluids, especially at meals. New agents for treatment of salivary hypofunction, such as pilocarpine hydrochloride or bromhexine, may be useful. Meticulous oral hygiene is essential, including regular flossing, brushing, and fluoride treatment at home and frequent dental checkups.
Instillation of artificial tears as often as every half hour prevents eye damage (corneal ulcerations, corneal opacifications) from insufficient tear secretion.
CLINICAL TIP: Artificial tears, whose drops are thicker and more viscous, require less frequent application but may cause blurring or leave residue on eyelashes.
Some patients may also benefit from instillation of an eye ointment at bedtime, or from twice-a-day sustained-release cellulose capsules.
If an eye infection develops, antibiotics should be given immediately; topical steroids should be avoided.
Other measures
Other treatment measures vary with associated extraglandular findings. Parotid gland enlargement requires local heat and analgesics; arthritis and arthralgias, hydroxychloroquine or nonsteroidal anti-inflammatory drugs; pulmonary and renal interstitial disease, corticosteroids; accompanying lymphoma, a combination of chemo-therapy, surgery, and radiation.
Special considerations
❑ 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, and tobacco, alcohol, and spicy, salty, or highly acidic foods, which cause mouth irritation. Adequate dental hygiene after meals can also decrease the frequency of dental caries.
CLINICAL TIP: Saliva flow can be stimulated by use of sugar-free, highly flavored lozenges (such as lemon drops).
❑ 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.
❑ To help relieve respiratory dryness, stress the need to humidify home and work environments. 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 using a vaginal lubricant.
❑ Refer the patient to the Sjögren’s Syndrome Foundation for additional information and support.
Book Source Details
- Book Title: Handbook of Diseases
- Author(s): Springhouse
- Year of Publication: 2003
- Copyright Details: Handbook of Diseases, Copyright © 2003 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Handbook of Diseases
Authors: Springhouse
Publisher: Lippincott Williams & Wilkins
Copyright: 2003
ISBN: 1-58255-266-5
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