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Sjogren's syndrome

†What is Sjogren's syndrome

Sjogren's syndrome is defined as a syndrome of xerophthalmia (dry eyes) and xerostomia (dry mouth) due to immune destruction of endocrine glands, especially of the lacrimal and salivary glands. Two types of Sjogren's disease

1. Primary Sjogren's syndrome- Sicca complex (dry eyes and dry mouth) and extra glandular symptoms without any additional connective tissue disorder

2. Secondary Sjogren's syndrome- Sicca complex that occurs with another autoimmune disorder such as SLE, RA, or scleroderma.

History of Sjogren's syndrome

Henrick Sjogren was first credited for the discovery of the disease. As a resident on ophthalmology, he discovered women with rheumatism and corneal abrasion who could not produce tears when crying. He later published a paper, Keratoconjuctiva Sicca, describing this dysfunction. His paper did not receive a good report. However, he later did a series of 80 patients with the syndrome, the majority having arthritis. After which, Sjogren's syndrome received attention.

Incidence of Sjogren's syndrome

1-2 million people in the US. Prevalence of 1-3% of the population. Sjogren's disease is in the top 3 of rheumatic diseases behind systemic lupus erythematosis and rheumatoid arthritis. Primary Sjogren's disease has a ratio of 9:1 of women to men. Age range is from 40-60 with mean age of 52.7 yrs. However case reports have been seen in children. Etiology Pathogenesis of Sjogren's disease is believed to multifactorial. Known to be autoimmune, but studies have suggested that the disease process has been genetic, environmental and neurodendocrine.

Prognosis and Treatment of Sjogren's syndrome

Sjogren's syndrome is slow in its course, as with all autoimmune diseases. Therapy is to treat symptoms. However, the risk of lymphomas is high, and a concern for mortality from the disease. Symptomatic treatments include artificial tears, tear and salivary stimulators, immunosuppressive drugs, surgical procedures, salivary substitutions, humidification, PPI. One must also have frequent dental visits to care for dental caries, and treat infection of bacterial and fungal regularly.

Pilocarpine was the first FDA approved anti-muscarinic therapy proved useful for stimulation of tears and saliva. A next generation anti-muscarinic therapy Evoxac is also being used. It has less of the cardiac side effect of muscarinic therapy.

The systemic effects of all autoimmune diseases such as muscle pain, renal impairment and pulmonary involvement benefit from use of steroids and other immune modulators. However, these therapies have been proven not to help with the sicca symptoms of Sjogren's disease.

In conclusion, Sjogren's disease has many early ENT manifestations that may be encountered first by otolaryngologist. It is therefore important that the practicing otolaryngologist be aware of this disease and be able to diagnosis the disease. The otolaryngologist is also an important part of the multidisciplinary team by providing biopsies and aiding in treatment

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