Sjogren’s Syndrome is a chronic disease characterized by dry eyes and mouth due to the immune destruction of the lacrimal and salivary glands.
It occurs as an isolated disorder, also known as sicca syndrome, more often associated with other autoimmune diseases such as arthritis, rheumatoid polymyositis, lupus, erythematosus, scleroderma, vasculitis, mixed connective tissue disease, or thyroiditis.
In Sjogren’s syndrome, there is a progressive infiltration by T lymphocytes with the loss of granular parenchyma, causing xerostomia, and xerophthalmia.
ETIOLOGY AND PATHOGENESIS
The characteristic reduction in tears and saliva is the result of lymphocytic infiltration and the fibrosis of the lacrimal and salivary glands. The infiltrate contains TCD4 + lymphocytes, lymphocytes, including plasma cells.
About 75% of patients have the rheumatoid factor, a reactive antibody to self-IgG. A large number of organ specific and non-specific antibodies have also been identified. The most important are antibodies directed against two antigens of the ribonucleoproteins, SS-A (Ro) and SS-B (La), that can be detected by up to 90% of patients with sensitive tests.
Although the pathogenesis of Sjogren’s Syndrome remains unclear, aberrant activation of T and B lymphocytes is involved. The initial trigger may be a viral infection of the salivary glands, which causes cell death and the release of self-tissue antigens.
Some viruses, notably the retrovirus and Epstein-Barr virus, have been implicated in the cause of Sjogren’s Syndrome.
The evidence suggesting a role for retroviruses comes from the demonstration of antibodies against HIV-related proteins in a subset of patients with Sjogren’s syndrome and from the clinical similarity of salivary gland disease secondary to HIV.
The presence of the Epstein-Barr virus can be demonstrated in the salivary gland tissue of patients with Sjogren’s syndrome. However, the virus is also found in the salivary glands of normal individuals and thus undermines the claim that this virus plays a primary role in causing this abnormality. If the Eptein-Barr virus does intervene, its role may be secondary in nature.
CLINICAL CHARACTERISTICS
It affects 0.5 – 1% of the population, although the disease is not inherited.
It is more common in women in their 50s and 60s.
Nasal dryness and crusting often develop, with ulceration and, infrequently, perforation of the nasal septum. As a consequence of respiratory involvement, laryngitis, bronchitis or pneumonitis may occur.
The sensation of dryness is very annoying, since the mucosa of the mouth and eyes is thin, inflamed, and irritated accompanied by a burning sensation. The dry mucosa is also very prone to candidiasis.
One of the main consequences of xerostomia is the frequency of root caries, generally in the amelocemental junction on the facial surface of the teeth.If disease affects only the salivary and lacrimal glands, without other systemic autoimmune manifestations, it is called Sjogren’s Syndrome. In patients with keratoconjunctivitis sicca, characterized by keratositic lesions, staining rose bengal,
keratoconjunctivitis produces blurred vision, burning and itching, while thick secretions accumulate in the conjunctival sac.
The Schirmer test is used to assess tear flow and reads positive if the flow is less than 5mm in a time of 5 minutes.
To distinguish the hypertrophy of the lacrimal and salivary glands caused by Sjogren’s Syndrome from other causes, it is useful to perform a lip biopsy to examine the minor salivary glands.
This technique is simple; it only requires a sample of six and eight minor salivary gland lobules, obtained from the lower lip. The lymphocyte clusters in the sample are counted to know the measurement by focus. The diagnosis is more likely if the count per focus exceeds that of normal glandular tissue.
Secondary Sjogren’s Syndrome occurs when there are other signs or symptoms of autoimmune disease, the most common being rheumatoid arthritis. Other collagen diseases may also occur, such as lupus erythematosus, systemic sclerosis, dermatomyositis, and mixed connective tissue disease.
Other primary or secondary diseases that occur are: Raynaud’s disease, interstitial nephritis, interstitial pneumonitis, purpura, and polymyopathy. There is also an increased risk of developing extrasalivary malignant lymphoma.
Some cases display glandular involvement. Tubulointerstitial nephritis, tubular atrophy occur frequently: renal tubular acidosis occurs with excess excretion of urate and phosphate.
There may be adenopathies with pleomorphic infiltrates in the lymph nodes. There is a 40 times greater risk of developing B-cell lymphoma in the affected glands.
In the laboratory tests used as an aid, we find contrast sialography, pertechnetate scintigraphy, and computed tomography that provide inflammatory images, rather than neoplastic ones. None of these imaging techniques is specifically diagnostic of Sjogren’s Syndrome since any sialadenitis can present the same characteristics.
TREATMENT
There is no effective treatment for this pathology. Unfortunately, the dryness progressively worsens, although some patients retain a certain degree of secretion. The complications of xerostomia are difficult to treat, although the patient can be helped to alleviate their discomfort while the development and progression of root caries can be minimized by applying daily topical fluoride gels and meticulous oral hydration.
Candidiasis can be treated with antifungal medications. You can use saliva or glycerin substitutes and lemon-flavored water, constantly taking small sips to relieve the feeling of dryness.
Pilocarpine and transmucosal stimulatory devices can stimulate secretion in patients who retain some functionality.
Secondary Sjogren’s syndrome, with its accompanying collagen diseases, will often require additional treatment including immunosuppressive agents.
All patients should receive regular dental care to avoid cavities and the tooth loss that arise as a complication of Sjögren’s syndrome. Patients with dry eyes should periodically see an ophthalmologist for signs of corneal damage. Patients with excessive redness and pain in the eyes should be evaluated for infection.
Hydroxychloroquine, an antimalarial drug used for lupus and rheumatoid arthritis, may be helpful in some patients with Sjögren’s syndrome, as it reduces joint pain and rashes in some cases. Patients with rare systemic symptoms such as fever, rashes, abdominal pain, or kidney or lung problems may need treatment with prednisone or immunosuppressive agents such as methotrexate, azathioprine, mycophenolate, or cyclophosphamide. In addition, researchers are evaluating rituximab and other biological therapies to treat Sjögren’s cases that affect the whole body.
CONTACT US
If you have any questions about this or other topics, contact us at Channel Island Family Dental, as well as on our Facebook page. At Channel Island Family Dental, we are always attentive to your needs to make a timely diagnosis. In addition, our dentists in Oxnard, Santa Paula, Newbury Park Ventura, and Port Hueneme will guide you to the best treatment to give you back your best smile.
