Neuropsychiatric complications developed in 40 patients with primary Sjögren's syndrome, none of whom met American Rheumatologic Association criteria for systemic lupus erythematosus. Twenty-five patients had psychiatric abnormalities, the commonest of which were affective disturbances. Of 30 patients tested, 23 had an abnormal pattern in the Minnesota Multiphasic Personality Inventory, the commonest pattern being a "conversion V." In general, patients presented with hysteroid dysphoric features. Of 16 patients undergoing cognitive function testing, 7 showed mild memory impairment with attention and concentration deficits. On clinical evaluation, 27 patients had neurologic abnormalities unattributable to other causes (central and peripheral nervous system in 16 and 19 patients respectively). There was a significant correlation between psychiatric disturbances and neurologic dysfunction, suggesting a possible organic basis for psychiatric dysfunction. The diagnosis of primary Sjögren's syndrome should be considered in patients with unexplained neuropsychiatric illness.

Two types of inflammatory vascular disease (IVD) occur in Sjogren's syndrome: neutrophilic IVD (NIVD) and mononuclear IVD (MIVD). In 45 patients with Sjogren's syndrome, we examined the 2 types of IVD with respect to serologic associations. NIVD, unlike MIVD, was significantly associated with seroreactivity reflected by hyperglobulinemia (P = 0.01), rheumatoid factor (P = 0.002), antinuclear antibodies (P = 0.02), and antibodies to Ro (SS-A) (P = 0.00006), and with hypocomplementemia (P = 0.03). The differential association of the 2 types of IVD with serologic reactivity and hypocomplementemia suggests that there may be basic differences in the immunopathogenesis of these 2 forms of IVD in Sjogren's syndrome.Inflammatory vascular disease (IVD) is increasingly being recognized as a complication of Sjogren's syndrome (SS) (1,2). Two distinct types of IVD have been observed (2): neutrophilic IVD (NIVD) and mononuclear IVD (MIVD). We previously reported a Publication #570 of the O'Neill Research Laboratories,

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Two histopathologic types of inflammatory vascular disease (IVD) occur in Sjogren's syndrome (SS):mononuclear IVD (MIVD) and neutrophilic IVD (NIVD). We describe 50 SS patients with IVD (30 with NIVD and 20 with MIVD). Thirty-three (66%) of the SS patients with biopsy-documented IVD had nervous system disease unattributable to other causes. Nineteen patients (58%) had involvement of both the central and peripheral nervous systems, while 9 had peripheral and 5 had central nervous system dysfunction alone. Patients with both histopathologic types of IVD were at risk for the development of nervous system abnormalities (57 % of NIVD patients and 80% of MIVD patients). Indirect evidence is presented which suggests that IVD may play a role in the immunopathogenesis of nervous system disease, at least in a subset of SS patients.