SJOGREN’S SYNDROME



SJOGREN’S SYNDROME

Sjogren’s syndrome is a systemic autoimmune disease characterized by dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). Sjogren’s syndrome can be a primary condition or it may be secondary to other autoimmune diseases including lupus, rheumatoid arthritis and scleroderma. It is not uncommon to encounter people with evidence of dry eyes or dry mouth, which may be considered sicca symptoms. In order to diagnose Sjogren’s syndrome a patient must have dry eyes, dry mouth and either a characteristic salivary gland biopsy or a positive SSA or SSB antinuclear antibody. Sjogren’s typically occurs in middle aged women, particularly of northern European ancestry. Ninety percent of cases are female. This condition was first described by Mikuliez in 1898 and was originally called Mikuliez syndrome. Sjogren again described the condition in 1933.

Ocular and Oral Symptoms

Patients suffer from decreased lubrication of the eyes due to a lymphocytic destruction of lacrimal glands. The decreased lubrication results in increased friction as the eyelids move over the sclera. The dry mouth is the result of lymphocytic infiltration of salivary glands. Initially there is a decrease in the basal secretion of these glands but with progression of disease there is a decrease in the secretion normally stimulated by food. The lack of saliva may lead to increased dental decay due to loss of salivary proteins, antibodies and mechanical removal of food particles. Dried mucous in the secretions from the parotid gland may plug up the duct resulting in painful swelling of the parotids. It is difficult to determine whether an incident of parotid swelling is due to infection or mechanical blockage or both. Patients often need to be treated with both antibiotics and corticosteroids for an attack.

Systemic Manifestations

Patients with Sjogren’s often have arthralgias or arthritis similar to rheumatoid arthritis. At times it may be difficult to distinguish whether a patient with rheumatoid arthritis has sicca symptoms or whether to classify all of their disease manifestations as Sjogren’s syndrome. Patients can also develop upper respiratory symptoms due to drying of the bronchial secretions or an autoimmune interstitial pneumonitis. Cardiac manifestations include pericarditis and pulmonary hypertension. Patients may develop renal involvement. In lupus the typical involvement is glomerulonephritis and in Sjogren’s it is more likely to be tubular involvement with interstitial nephritis. Sjogren’s syndrome patients manifest neurological involvement with peripheral neuropathies and occasional CNS events such as strokes. It is not uncommon to see scattered abnormalities in the white matter on MRI scan of asymptomatic patients with Sjogren’s.

Laboratory Findings

Approximately 80% of Sjogren’s patients have a positive antinuclear antibody. In addition 60 to 80% of patients have a positive SSA or SSB antibody. Unfortunately the SSA and SSB are also seen in approximately 30% of patients with lupus without any overt evidence for Sjogren’s syndrome so these are not entirely specific. There are several methods for objectively measuring eye involvement in Sjogren’s syndrome. You can perform a staining of the cornea with Rose Bengal stain, which will reveal on ophthalmologic exam superficial erosions of the cornea. You can also check a Schirmer’s test with a small strip of test paper placed on the conjunctiva for five minutes. There should be greater than 8mm of wetting of the strip in normal subjects. You can objectively evaluate dry mouth by checking parotid flow rate using a Lashley cup or sending the patient to the ENT for a minor salivary gland biopsy. In addition drainage of the parotid gland can be evaluated by a MR angiogram. Patients with Sjogrens commonly exhibit a mild neutropenia.

Treatment

The initial treatment of sicca symptoms includes artificial tears. There are a variety of products available as artificial tears which offer a few hours of relief per application. Another approach is to plug the tear ducts. For nighttime there is lubricating ointments or gels available to offer longer duration of relief, however these cause blurring during the daytime since they are thick.

Management of the dry mouth is more difficult. There are a number of products available over the counter including salivart spray or mouth kote. One option is to use vaginal lubricants, which are inexpensive and available over the counter. These have been tested by the FDA for oral safety despite being marketed as vaginal lubricants. Another issue is the use of fluoride to help retard dental decay. Patients should be seen regularly by a dentist who is familiar with Sjogren’s syndrome to help prevent permanent tooth damage. Refer patients to internet support groups for OTC product suggestions.

Pilocarpine (Salagen) increases parasympathetic stimulation, which results in SLUD (salivation, lacrimation, urination, and defecation). This agent is effective for relieving the salivary and ocular symptoms of patients. However, patients also suffer from bladder and bowel symptoms as well as excess perspiration or dizziness with these agents. If a patient has severe sicca symptoms it is worth trying Salagen 5 mg t.i.d. or a newer product, Evoxac, which is usually a little better tolerated but is more expensive.

The utility of other immunosuppressive drugs are unclear in Sjogren’s syndrome, though not well tested. It may be beneficial to use Plaquenil to try and avoid some of the autoimmune sequelae of this disease. It is well established to use prednisone, Imuran or Cytoxan for severe CNS or renal manifestations, but this is based on experience with lupus, not on studies. For patients with significant arthritis methotrexate or leflunomide may be useful.

Lymphoma

Sjogren’s patients are at risk for lymphoma. Patients can develop severe lymphadenopathy should be biopsied – they may develop pseudo lymphoma, which may eventually develop into lymphoma.

TB 2016

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