THE EUROPEAN-COMMUNITY STUDY-GROUP ON DIAGNOSTIC-CRITERIA FOR SJOGRENS-SYNDROME - SENSITIVITY AND SPECIFICITY OF TESTS FOR OCULAR AND ORAL INVOLVEMENT IN SJOGRENS-SYNDROME

Aim-To establish a definitive set of diagnostic criteria in a multicen tre European study a selected number of oral and ocular tests were per formed on a large number of patients with Sjogrens Syndrome (SS) and c ontrols. The diagnostic accuracy of each test far patients with primar y and secondary SS and for controls at different ages, was studied. Me thods-Each centre received a clinical chart describing the series of t ests to be conducted. The tests included: questionnaires for dry eye a nd dry mouth symptoms, Schirmer's-I-test (ScT), tear fluid lactoferrin level (TFLL), break-up time (BUT) and rose Bengal score (RBS) for the eye evaluation; unstimulated and stimulated whole saliva collection ( UWSC and SWSC), salivary gland scintigraphy (SGS), parotid sialography (PS) and minor salivary gland biopsy (MSGB) for oral involvement. Res ults-Data from 22 centres and 11 countries was collected on a total of 447 patients with SS (246 with primary SS and 201 with secondary SS) and 246 controls (of whom 113 had a connective tissue disease without SS). Among the ocular symptoms, the feeling of dry eye and 'sand in th e eye' were the ones most commonly recorded in patients with SS. Simil arly, the feeling of dry mouth, appearing either spontaneously or when the patient was eating or breathing, was the most frequent subjective oral symptom. Among the ocular tests, ScT showed the best balance bet ween sensitivity and specificity (76.9% and 72.4% respectively), while RBS was the most specific test (81.7%). ScT and RBS gave also suffici ently concordant results. TFLL and BUT gave considerably less reliable results, which were not concordant with each other or with the other ocular tests. The quantitative lacrimal tests ScT and TFLL produced si gnificantly different results in elderly controls, while RBS did not. Abnormal results for all of the ocular tests were less marked and less frequent in patients with secondary SS than in those with primary SS. The oral tests (except SWSC) were generally more reliable than the oc ular tests in diagnosing SS. In particular, PS was the most specific d iagnostic tools (100%), while MSGB (where the presence of at least one inflammatory focus was considered as indicative for the diagnosis) sh owed a good balance between sensitivity and specificity (82.4% and 86. 2%, respectively). The tests showed a good degree of agreement and, wi th the exception of UWSC, were not influenced by age. In the oral, as in the ocular tests, abnormal results were less frequent and less mark ed in patients with secondary SS.Conclusions-The results clearly show that ScT and RBS (for the eye evaluation), and SGS, PS, MSGB and UWSC (for salivary gland involvement) are the most reliable tests for the d iagnosis of SS. The clinician should be aware, however, that the test results may vary depending on the age of the patient and the type of S S (primary or secondary).