Sjogren's syndrome (SS) is a chronic inflammatory disease associated with lymphocytic infiltration of exocrine glands. Certain clinical, serologic, and genetic differences are found among patients with SS alone, also called primary SS (patients with keratoconjunctivitis and xerostomia in the absence of any other definable connective tissue disease). These differences suggest varying etiologies for similar clinical manifestations of disease. The most commonly accepted definition of SS has been the presence of two of the following findings: (a) keratoconjunctivitis sicca (dry eyes), (b) xerostomia (dry mouth), and (c) one of the connective tissue disease syndromes. Secondary SS is defined as keratoconjunctivitis and xerostomia as features of a connective tissue disease. Rheumatoid arthritis (RA) is the most common connective tissue disease seen in association with secondary SS, but others have been well-documented and include systemic lupus erythematosus (SLE), scleroderma, polymyositis, mixed connective tissue disease, and juvenile rheumatoid arthritis. As a result of the obvious shortcomings of the above, purely clinical definition, most recent investigators have included pathologic criteria (see section III) as supporting evidence of the presence of primary or secondary SS.
The clinical manifestations of the disease were first described in 1888. In 1933, Henrik Sjogren published a monograph describing in great detail the histologic and clinical components of this syndrome, which has often been referred to as Sjogren's syndrome. Confusion later arose between SS and the disease previously reported by Mikulicz in the late 1800s. In 1927, Schaffer and Jacobsen defined two main categories: (a) Mikulicz's disease proper, of unknown etiology and following a benign course, and (b) Mikulicz's syndrome, caused by a variety of disorders such as leukemia, lymphosarcoma, tuberculosis, sarcoidosis, and iodide poisoning. Later, Morgan and Castleman concluded, on the basis of pathologic descriptions, that Mikulicz's disease and SS were identical. Much of the interest in SS during the past decade has focused on the opportunity to study the interrelationships among the autoimmune disorders, lymphoproliferative malignancies, and dysproteinemias, all of which may be features of the syndrome.
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