SJÖGREN'S SYNDROME

Abstract

Sjogren's syndrome is a chronic systemic inflammatory disorder characterized by the presence of dry eyes and dry mouth. Dryness in the eyes and mouth are secondary to diminished glandular production secondary to lymphocytic and plasma cell infiltration. Because of the prominent autoimmune features of this condition, Sjogren's syndrome has recently been referred to as an autoimmune exocrinopathy. 32 Although there is no universally accepted definition of Sjogren's syndrome, the diagnostic triad of keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth) associated with the presence of a connective tissue disorder is commonly employed. 7 Dry eyes may occur with or without lacrimal gland enlargement. Dry mouth may occur with or without salivary gland enlargement. Both primary and secondary forms of this disorder are recognized. Although primary Sjogren's syndrome may be a distinct disorder in the absence of another known connective tissue disease, the most frequent association of Sjogren's syndrome and a connective tissue disorder is with rheumatoid arthritis. Approximately one half of all patients with rheumatoid arthritis will present with Sjogren's syndrome whereas a less frequent association is seen with less common connective tissue disorders such as systemic lupus erythematosus or scleroderma. 30,49 It is important to distinguish these patients from patients that present with sicca complex, which includes only the presence of ocular and oral dryness. Because patients with primary sicca do not appear to have the systemic disease associations that are discussed in this article, they clearly would have a more favorable prognosis. Historically, features of Sjogren's syndrome have been recognized for over a decade. In 1892, Mikulicz reported the findings of bilateral parotid and lacrimal gland enlargement associated with significant round cell inflammation. 24 Although numerous reports appeared between 1883 and 1933 regarding patients with Mikulicz's syndrome, it was not until 1933 that Heinrich Sjogren described the syndrome that bears his name. 31 It is now generally accepted that Sjogren's syndrome and Mikulitz's disease are in fact variants of the same disorder. 25 It is important for the clinician to consider the diagnosis of Sjogren's syndrome in a patient who presents with suspected allergic disease. Patients with Sjogren's syndrome may present not only with dry eyes and dry mouth but may have prominent respiratory and ocular manifestations that are suspected to be of allergic origin. Because Sjogren's syndrome is suspected to affect 2 to 3 million individuals in the United States alone, 46 it is considered to be in fact the most common autoimmune disease process. Given its reasonably frequent occurrence and its potential for associated lymphoid malignancies, it is crucial that the clinician be aware of the manifestations of this disorder.