Sjorgen's syndrome

 

• symptoms and signs
• laboratory findings
• about the disease
• prevention
• treatment
• prognosis

Symptoms and signs. burning, itching, ropy secretions in eyes, with impaired tear production during crying (keratoconjunctivitis sicca); parotid enlargement develops in one-third of patients; dryness of mouth (xerostomia) leads to difficulty in speaking and swallowing and dental caries; may have loss of taste and smell; desiccation may involve nose, throat, larynx, bronchi, vagina and skin; systemic manifestations include dysphagia, pancreatitis, pleuritis, neuropsychiatric dysfunction and vasculitis

Laboratory findings. mild anemia, leukopenia and eosinophilia; rheumatoid factor found in 70% of patients; increased levels of gamma globulins and antinuclear antibodies; presence of anti SS-A and SS-B (cytoplasmic antigens); Schirmer’s test to measure quantity of tears secreted; radionuclide scanning to evaluate salivary gland; lymphoid foci in labial biopsy for confirmation of disease; biopsy of parotid gland if unilaterally enlarged

About the disease. Sjogren's syndrome is an autoimmune disorder resulting from chronic dysfunction of exocrine glands in many areas of the body. It is characterized by dryness of the eyes, mouth and other areas covered by mucous membrane and is frequently associated with a rheumatic disease, most often rheumatoid arthritis. It is predominantly a disease of women, in a ratio of 9:1, with greatest incidence between age 40 and 60 years. Sjogren’s syndrome is frequently associated with the several disorders including: rheumatoid arthritis, systemic lupus erythematosus, primary biliary cirrhosis, scleroderma, polymyositis, Hashimoto’s thyroiditis, polyarteritis, and interstitial pulmonary fibrosis. When Sjogren’s syndrome occurs without rheumatoid arthritis, HLA-DR2 and HLA-DR3 antigens are present with increased frequency. Renal tubular acidosis occurs in 20% of patients. Chronic interstitial nephritis, which may result in impaired renal function, may be seen. A spectrum of lymphoproliferation ranging from benign to malignant may be found. Malignant lymphoma and Waldenstrom’s macroglobulinemia occur four times more frequently than normal.

Prevention. no known preventive measures.

Treatment. symptomatic and supportive; application of artificial tears to relieve ocular symptoms; lubricate mouth; avoid atropine drugs and decongestants (will decrease salivary symptoms); oral hygiene to preserve dentition; treat associated rheumatic disease, if present.