ASSESSMENT OF THE EUROPEAN CLASSIFICATION CRITERIA FOR SJOGRENS-SYNDROME IN A SERIES OF CLINICALLY DEFINED CASES - RESULTS OF A PROSPECTIVEMULTICENTER STUDY

Objective-To assess the recently proposed preliminary criteria for the classification of Sjogren's syndrome (SS) in a multicentre European s tudy of a new series of clinically defined cases. Methods-The criteria included six items: I = ocular symptoms; II = oral symptoms; III = ev idence of keratoconjunctivitis sicca; IV = focal sialoadenitis by mino r salivary gland biopsy; V = instrumental evidence of salivary gland i nvolvement; VI = presence of autoantibodies. Each centre was asked to provide five patients with primary SS, dive with secondary SS, five wi th connective tissue diseases (CTD) but without SS, and five controls (patients with ocular or oral features that may simulate SS). The prel iminary six item classification criteria set was applied to both the S S patients and the non-SS controls, and the performance of the criteri a in terms of sensitivity and specificity was tested. Results-The crit eria set was tested on a total of 278 cases (157 SS patients and 121 n on-SS controls) collected from 16 centres in 10 countries. At least fo ur of the six items in the criteria set (Limiting item VI to the prese nce of Ro(SS-A) or La(SS-B) antibodies) were present in 79 of 81 patie nts initially classified as having primary SS (sensitivity 97.5%), but in only seven of 121 non-SS controls (specificity 94.2%). When the pr esence of item I or II plus any two of items III-V of the criteria set was considered as indicative of secondary SS, 97.3% (71 of 73) of the patients initially defined as having this disorder and 91.8% (45 of 4 9) of the control patients with CTD without SS were correctly classifi ed. Conclusion-This prospective study confirmed the high validity and reliability of the classification criteria for SS recently proposed by the European Community Study Group.