Sjögren's syndrome. Proposed criteria for classification

Abstract

The term “Sjögren's syndrome” (SS) is frequently used to describe the occurrence of keratocon-junctivis sicca and xerostomia in association with an autoimmune disorder. However, well-defined criteria for the classification of SS have not been established, and this diagnosis is being applied to a wide spectrum of conditions, ranging from clear “autoimmune” disease in some patients, to sicca complaints without evidence of a systemic immune process in elderly patients. Here, we review the clinical and laboratory features of patients referred for evaluation of sicca symptoms. In particular, we emphasize the need for care in choosing the site for minor salivary gland biopsy, and we describe the histologic features that aid in the evaluation of these biopsy specimens. In an attempt to identify a population of patients whose conditions might have a common etiopathogenesis and, thus, a common treatment program, we propose the following criteria for a diagnosis of SS: 1) objective evidence of keratoconjunctivis sicca, as documented by rose bengal or fluorescein dye staining; 2) objective evidence of diminished salivary gland flow; 3) minor salivary gland biopsy, obtained through normal mucosa, with the specimen containing at least 4 evaluable salivary gland lobules, and having an average of at least 2 foci/4 mm²; 4) evidence of a systemic autoimmune process, as manifested by the presence of autoantibodies, such as rheumatoid factor and/or anti-nuclear antibody. The diagnosis of “definite SS” would be made when all 4 criteria are met; the diagnosis of “possible SS” would be made when 3 criteria are present. Specific exclusions for this diagnosis are preexisting lymphoma, graft-versus-host disease, sarcoidosis, and acquired immunodeficiency disease. These proposed criteria are more restrictive than are those currently used for diagnosing SS. Future multicenter trials will be required to determine whether these criteria are generally useful. Finally, we emphasize the rheumatologist's role in selecting safe, inexpensive methods of objectively monitoring sicca complaints (i.e., kerato-conjunctivitis sicca and parotid salivary flow) and in evaluating the results of minor salivary gland biopsy.