Mileposts in the Understanding of Sjögren's Syndrome
First described in the late nineteenth century, xerostomia, or dry mouth, is the result of salivary gland hypofunction. In 1925, Gougerot recognized a generalized condition that produced dryness of the eyes, mouth, larynx, nose, and vulva.[1] In 1933, Swedish ophthalmologist Henrik Sjögren described a cluster of 19 patients suffering from dry eyes and mouth, 70% of whom presented with arthritis. Microscopic examination of specimens obtained from these patients revealed extensive chronic inflammatory cell infiltrates in the lacrimal, parotid, sublingual, and labial salivary glands.[1]
In 1965, Sjögren's syndrome (SS) was defined as "the triad of keratoconjunctivitis sicca (dry eyes), xerostomia (dry mouth), and rheumatoid arthritis or other connective tissue disease."[2] Today, the terms primary and secondary SS are widely accepted and used to categorize the syndrome. Primary SS is the diagnosis for individuals who have ocular dryness and xerostomia only.The term secondary SS is used when connective tissue disease also is present. The connective tissue disease most commonly found in patients with secondary SS is rheumatoid arthritis (which occurs in about half of SS patients) (Fig. 1). However, systemic lupus erythematosus, progressive systemic sclerosis (scleroderma), mixed connective tissue disease, polymyositis/dermatomyositis, biliary cirrhosis, or polyarteritis nodosa may present.
Figure 1. Rheumatoid arthritis. Female patient with classic signs of rheumatoid arthritis on her right hand.
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