Sjogren’s Syndrome is the expression of an autoimmune process that results principally in dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) owing to lymphocytes (a types of white blood cell) mediated destruction of lacrimal (tear) and salivary glands parenchyma (the functional tissue of an organ as distinguished from the connective and supporting tissue). Other autoimmune conditions, particulary rheumatoid arthritis*, lupus erythematosus**, and scleroderma***, may also be seen as a component of this syndrome. Lacrimal and salivary gland involvement is often one expression of this generalized exocrinopathy (problem in exocrine glands of where they secrete substances outside the body) that is lymphocyte mediated.
Syndrome is a collection of symptoms.
*Rheumatoid Arthritis is a chronic progressive disease causing inflammation in the joints and resulting in painful deformity and immobility, especially in the fingers, wrists, feet, and ankles.
**Lupus erythematosus is an autoimmune inflammatory disease causing scaly red patches on the skin, especially on the face, and sometimes affecting connective tissue in the internal organs.
***Scleroderma is a chronic hardening and contraction of the skin and connective tissue, either locally or throughout the body.
This syndrome appears to be of autoimmune origin that may be limited to exocrine glands, or it may extend to include systemic connective tissue disorders. In instances of only exocrine glandular involvement, the syndrome is known as primary Sjogren’s syndrome. If an associated connective tissue disorder is present, in addition to the xerostomia and keratoconjunctivitis sicca, regardless of the specific type, it is known as seconday Sjogren’s syndrome.
Examples of connective tissue disorders are like rheumatoid arthritis, lupus erythematosus, & scleroderma.
- Clinical Features
Sjogren’s syndrome occurs in all ethnic and racial groups. The peak age of onset is 50 years, and 90% of cases occur in women. Children and teenagers are rarely affected. Distinguishing between primary and secondary forms of this syndrome, especially those associated with rheumatoid arthritis, usually is not difficult. This may be important because of increased risk that lymphoreticular malignancy* may develop in the primary form in approximately 5% of the patients. Lymphoma** is considered a late development during the course of the syndrome, occurring from 7.5 to 14 years after disease onset. Often presaging or predicting the development of malignancy are parotid*** enlargement, lymphadenopathy****, inflammatory neuropathy*****, and vasculitis******.
*Lymphoreticular malignancy is a disease of the lymphoreticular system commonly associated with cell-mediated immune deficiencies in which patients have a scarcity of normal white blood cells.
**Lymphoma is a cancer of the lymph nodes.
***Parotid enlargement is an enlargement of parotid salivary gland just in front of the ear on the lateral side of the face.
****Lymphadenopathy is a disease affecting the lymph nodes.
*****Inflammatory neuropathy is a condition is characterized by pain, fatigue, numbness, and weakness, especially in extremities.
******Vasculitis is inflammation of a blood vessel.
The chief oral complaint in Sjogren’s syndrome is xerostomia (dry mouth), which may be the source of eating and speaking difficulties. These patients are also at greater risk of dental caries, periodontal disease, and oral candidiasis (fungal infection of mouth) caused by dry mouth. Parotid gland enlargement, which is often recurrent and symmetric, occurs in approximately 50% of patients (Figure 1).
A significant percentage of these patients also present with complaints of arthralgia, myalgia, and fatigue.
Other laboratory findings commonly found in primary and secondary Sjogren’s syndrome include mild anemia, leukopenia (low white blood cells), eosinophilia (a type of white blood cell which is available in low), an elevated ESR (inflammatory marker), and diffuse elevation of serum immunoglobulin levels. In addition, numerous autoantibodies may be found, including rheumatoid factor, antinuclear antibodies, and precipitating antinuclear antibodies such as anti-Sjogren’s syndrome-A (SS-A) and anti-Sjogren’s syndrome-B (SS-B). Antibodies SS-A and SS-B may be seen in association with both primary and secondary Sjogren’s syndrome. Patients who have SS-B antibodies are more likely to develop extraglandular disease.
Diagnosis depends on the correlation between patient history and laboratory data, clinical examination, and assessment of salivary gland function. An important consideration concerns the clinical manifestation of xerostomia (dry mouth). Although this is the main oral symptom and clinical sign in Sjogren’s syndrome, other considerations of dry mouth must be evaluated. In addition, major salivary gland enlargement is a feature of Sjogren’s syndrome, but it may be episodic in nature and may not be present at all in some patients.
Diagnosis may involve lip biopsy for salivary component, Schirmir test and wafer test for lacrimal component (tears), serologic tests for rheumatoid factor, Antinuclear antibody (ANA), Sjogren’s syndrome – A (SS-A), Sjogren’s syndrome (SS-B).
Sjogren’s syndrome and the complication of the sicca (dry) component are best managed symptomatically. Artificial saliva and oral lubricants as well as artificial tears are available for this purpose. Preventive oral measure are extremely important relative to xerostomia. Scrupulous oral hygiene, dietary modification, topical fluoride therapy, and remineralizing solutions are important in maintaining oral & dental tissues. Use of sialogogues, such as pilocarpine and cevimeline, remains of limited value, especially in long-standing Sjogren’s syndrome. Dietary considerations also are important, whereby the patient must avoid intake of caffeine-containing drinks and foods and limit consumption of cariogenic foods and drinks.
The prognosis of Sjogren’s syndrome is complicated by an association with malignant transformation to lymphoma. This may occur in approximately 5% of cases, but it is more common in those with only the sicca component of the syndrome (primary Sjogren’s syndrome).
Generally, the course for Sjogren’s syndrome is one of chronicity, requiring long-term symptomatic management. careful follow-up and management by a dentist, ophthalmologist, and rheumatologist, among others, are critical. In cases of severe primary Sjogren’s syndrome with systemic complications, a promising treatment option is the use of anti-CD20 monoclonal antibody (rituximab), although this remains an open issue in the absence of randomized controlled trials.
Reference: Oral Pathology, Sixth Edition, By Regezi, Sciubba, & Jordan.
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Haider Maitham, DDS