Objective. Different sets of diagnostic criteria have been proposed for Sjogren's syndrome (SS), but none have been validated with a large series of patients or in a multicenter study. We conducted the present study involving 26 centers from 12 countries (11 in Europe, plus Israel), with the goals of reaching a consensus on the diagnostic procedures for SS and defining classification criteria to be used in epidemiologic surveys and adopted by the scientific community.Methods. The study protocol was subdivided into two parts. For part I, questionnaires regarding both ocular and oral involvement were developed; they in-

Objective-To assess the recently proposed preliminary criteria for the classification of Sjogren's syndrome (SS) in a multicentre European study of a new series of clinically defined cases. Methods-The criteria included six items: I = ocular symptoms; II = oral symptoms; II = evidence of keratoconjunctivitis sicca; IV = focal sialoadenitis by minor salivary gland biopsy; V = instrumental evidence of salivary gland involvement; VI = presence of autoantibodies.

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Objective. Fcy receptors of class IIa (FcyRIIa) occur in 2 allelic forms, with either a low (IIa-R131) or a high (IIa-H131) affinity for complexed IgG2 and IgG3. This polymorphism might have implications for the handling of immune complexes. Therefore, we determined the distribution of the Fc yRIIa allotypes in patients with systemic lupus erythematosus (SLE), with or without a history of lupus nephritis.Methods. We studied 95 unrelated white European patients with SLE, as defined by the American College of Rheumatology criteria, 50 of whom had a history of lupus nephritis, and 69 healthy white European control subjects. Fc yRIIa allotypes were determined by immunophenotyping of blood monocytes.Results. It was found that lupus nephritis was significantly associated with the "low affinity" FcyRIIa WR131 allotype and with the R131 allele, compared with healthy controls. No significant association was found upon comparison of groups with and without nephritis.Conclusion. SLE patients with a history of lupus nephritis have an abnormal distribution of FcyRIIa allotypes. FcyRIIa may well play a role in the pathogenesis of lupus nephritis, since IIa-R/R131 SLE pa-

Objective-Although deforming arthropathy in systemic lupus erythematosus (SLE) is characterised by a number of manifestations, definitive criteria for the diVerent forms have not yet been established. To define deforming arthropathy and its diVerent types a study was undertaken of 176 SLE patients. Methods-Using as criterion any deviation from any of the metacarpus finger axes 17 patients (16 women, one man) were identified with clinical deforming arthropathy. These patients were evaluated according to a standardised protocol that covered all known characteristics of deforming arthropathy. By means of "Jaccoud's arthropathy index" three diVerent forms were identified. Results-Three patients had an erosive form of deforming arthropathy (or rhupus hand) such as those seen in frank rheumatoid arthritis (RA), eight patients were identified as having Jaccoud's arthropathy (or lupus hand), and the remaining six patients had mild deforming arthropathy. Jaccoud's arthropathy is characterised by severe deformation of the hands (ulnar deviation, swan neck deformities, and Z deformity of the thumb) and feet with multiple non-erosive subluxations, mild aching and little or no evidence of synovitis. All patients, but one, fulfilled just four criteria of the ACR classification and joint symptoms were always found to precede the diagnosis of SLE. Furthermore a remarkable association of Jaccoud's arthropathy with fetal loss, thrombosisboth venous and arterial-and the presence of antiphospholipid antibodies was found. Conclusions-These data suggest that Jaccoud's arthropathy represents a subset of SLE. Subdivision of deforming arthropathy into several clinical forms can facilitate the clinical management of this disorder. (Ann Rheum Dis 1998;57:540-544)

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