Have you noticed that your mouth is often dry? Or that your eyes feel like they have sand in them? If so, you may be suffering from an autoimmune disorder that affects more people than rheumatoid arthritis and that often goes undetected.
The disorder, Sjogren's syndrome (SS), occurs in 1-3% of people, yet SS remains undiagnosed more than half of the time. Women are affected much more often than men (ratio of 9:1). Though the syndrome can appear at any age, Sjogren's occurs primarily among women in their thirties or forties.
It is important to establish a diagnosis of SS for a number of reasons because treatment can improve an unsuspecting patient's quality of life. And, for a small group of patients with the disorder, who may be at an increased risk for the lymphoma, a malignant cancer, early diagnosis is especially important.
- Glandular, which result from destruction of glands by an invasion of lymphocytes (white blood cells)
- Systemic, which are characterized by lymphocyte invasion of the skin, lungs, kidneys or peripheral nervous system.
- Xerostomia (dry mouth)
- Parotid gland enlargement
- Xerophthalmia (dry eyes)
- Xerotrachea (dry trachea)
- Dyspareunia (painful sexual intercourse)
- Raynaud's phenomenon, easy bruising (purpura), recurrent hives (urticaria)
- Lung involvement
- Difficulty swallowing food
- Inflammation of the kidneys (interstitial nephritis)
- Peripheral neuropathy
On exam, the affected patient has little or no saliva in the floor of their mouth; in advanced disease, the surface of the tongue becomes red and lobulated, with partial or complete depapillation.
Normally, the salivary glands produce 1-1.5 L of saliva every day, which help fight bacteria, so that, in its absence, patients with SS are prone to develop dental carries and increased rate of oral bacterial infections with Streptococcus mutans and Lactobacillus. Furthermore, SS is the most common cause of acute bacterial infection of the salivary glands (sialadenitis). Acute bacterial sialadenitis produces a tender swelling of the salivary gland and, sometimes, other lymph node enlargement, fever and malaise.
Dry mouth, however, is not specific for SS and can be found in a number of other conditions as listed in Table 2.
|D/D of dry mouth||D/D of parotid gland enlargement||D/D of dry eyes|
Parotid gland enlargement:The parotid gland, a salivary gland, is located below and in front of each ear, and is often enlarged, occurring in 60% of patients with pSS. It may appear once in a while or as chronic persistent enlargement. Parotid gland enlargement may present on one side only (unilateral) but usually affects both parotid glands (bilateral).
Xerophthalmia(destruction of the lacrimal glands resulting in dry eyes): This is the second of the two subjective criteria for the diagnosis of SS. A broader term for this condition is "keratoconjunctivitis sicca," denoting the clinical findings that result from destruction of covering protective eye tissue whose function it is to bathe the eye.
Patients with xerophthalmia will complain of itching, burning and a sensation of gravel or sand in their eyes. They may also complain of redness of their eyes, photosensitivity, eye fatigue and a discharge from their eyes. These symptoms are made worse by dry climates and air-conditioned environments or with exposure to cigarette smoke. Untreated, xerophthalmia may be complicated by corneal ulceration, vascularization, opacification and, more rarely, corneal perforation.
- Ocular symptoms: persistent dry eyes (>3 months)
- Oral symptoms: persistent dry mouth
Four Objective Criteria:
- Signs of dry eyes: positive Shirmer's test or positive Rose Bengal score upon ophthalmologic evaluation
- Histopathology of lip biopsy: minor salivary glands with lymphocytic infiltrates
- Presence of either Ro or La autoantibodies in the serum
- Objective measurements of decreased salivary gland function (e.g., less saliva)
- Hepatitis C infection
- Pre-existing lymphoma
- Past history of head and neck irradiation
- Graft vs. host disease
- Use of anti-cholinergic drugs
The presence of any 4 out of 6 criteria is indicative of the diagnosis of pSS with a high degree of certainty (sensitivity of 97% and a specificity of 89%). If 3 out of the 4 objective criteria are present, then pSS can be diagnosed with a sensitivity of 84% and a specificity of 95%, even if the patient doesn't complain of dryness.
sSS is diagnosed when 1 of the 2 subjective and 2 of the 4 objective criteria are present in a patient with another well-defined connective tissue disease. These criteria and the studies that validated them stress the importance of objective blood tests for specific antibodies (Ro and La autoantibodies) and histopathology studies. Histopathology refers to the examination of minor salivary glands that are removed with a small (~1cm) incision of the skin (biopsy) overlying the lower lip. The size and number of white blood cells groupings that are adjacent to salivary ducts are counted. It is important to remember that other diseases such as sarcoidosis and AIDS (HIV sialadenitis) may look the same &8212; so it's extremely important that an experienced pathologist to interpret the biopsy and keep in mind the full clinical picture of the patient.
- Xerostomia is treated with drugs know as muscarinic agonists. Two are now available in the U.S.: pilocarpine hydrochloride, which is dosed as 5 mg tablets 4 times daily, and cevimeline hydrochloride, given as 30 or 60 mg tablets three times daily. Side effects may include excessive sweating (less often with cevimeline), nausea, inflammation of the nasal passages (rhinitis) and diarrhea. Frequent dental visits are advised to prevent and treat dental carries and oral infections. Patients with SS should avoid, if possible, tricyclic antidepressants, beta blockers and diuretics, since these may worsen their symptoms.
- Xerophthalmia is treated primarily with topical hydrophilic solutions to prevent complications such as corneal ulceration. Blocking the outflow ducts in the eyes, through the insertion of plugs, or permanent occlusion, through electrocautery, are also employed to preserve existing tears. Pilocarpine and cevimeline may also be used for xerophthalmia. Topical cyclosporine A has recently been reported as effective for xerophthalmia. Treatment of systemic manifestations is usually centered on non-aggressive symptomatic treatment with non-steroidal anti-inflammatory drugs (NSAIDs) or even low-dose corticosteroids or hydroxychloroquine for arthralgias. Long-term immunosuppression has not been shown to be of benefit and is hazardous, especially since the syndrome usually does not carry an increased mortality. Moderate doses of corticosteroids may be used in patients who have severe skin lesions or who have kidney disease.