Assessment oftheEuropean classification criteria forSjogren's syndrome inaseries ofclinically defined cases: results ofaprospective multicentre study

Objective-To assesstherecently proposedpreliminary criteria fortheclassification ofSjogren's syndrome(SS)ina multicentre Europeanstudyofa new series ofclinically defined cases. Methods-Thecriteria included sixitems: I=ocular symptoms;II=oralsymptoms; II=evidence ofkeratoconjunctivitis sicca; IV=focalsialoadenitis byminorsalivary gland biopsy; V=instrumental evidence of salivary glandinvolvement; VI=presence ofautoantibodies. Eachcentre wasasked toprovide five patients withprimarySS, fivewithsecondary SS,fivewithconnective tissue diseases (CTD)butwithout SS,andfive controls (patients withocular ororalfeatures thatmay simulate SS). Thepreliminary sixitemclassification criteria setwas applied toboththeSS patients andthenon-SScontrols, andthe performance ofthecriteria intermsof sensitivity andspecificity wastested. Results-The criteria setwastested ona total of278cases(157SSpatients and121 non-SScontrols) collected from16centres in10countries. Atleast fourofthesix itemsinthecriteria set(limiting itemVI tothepresence ofRo(SS-A) orLa(SS-B) antibodies) werepresent in79of81patients initially classified ashavingprimarySS (sensitivity 97.5%), butinonlysevenof121 non-SScontrols (specificity 94.2%). When thepresence ofitemIorIIplusanytwo ofitemsIll-V ofthecriteria setwasconsidered as indicative ofsecondary SS, 97-30/o (71of73)ofthepatients initially defined ashaving thisdisorder and91.8% (45of49)ofthecontrol patients withCTD without SSwerecorrectly classified. Conclusion-This prospective study confirmed thehighvalidity andreliability of theclassification criteria forSSrecently proposed by theEuropeanCommunity StudyGroup.