To evaluate nervous system involvement in a cohort of Italian patients with primary Sjögren's syndrome (pSS), 87 unselected patients (83 female, and four male) observed consecutively at our institution over a period of 5 years were screened by clinical and instrumental (MRI, SPECT, electrophysiological testing, CSF analysis) investigations for peripheral and central neurological abnormalities. Seroimmunological parameters and extraglandular features other than neurological manifestations were also evaluated. Seven patients had central nervous system (CNS) disease (8%), mostly non-focal dysfunction, and 12 had peripheral nervous system (PNS) disease (13.8%), mostly mild or severe sensory or sensory-motor polyneuropathies. One patient had concomitant CNS and PNS involvement. Compared with CNS disease, PNS involvement occurred in older patients (> 50 years), independent of the disease duration. Patients with and without neurological abnormalities did not differ for seroimmunological parameters (including antiphospholipid antibodies) or extraglandular manifestations. From a statistical point of view, the only relevant finding was the detection of a slight increase in serum IgA and IgM levels (p < 0.05) in patients with an intact nervous system. Neurological involvement in pSS, be it central or peripheral, is not a rare finding. A careful clinical neurological evaluation, combined with a multiplicity of instrumental investigations, is recommended in the global assessment of pSS patients.

To compare the clinical, laboratory and immunological features of a group of Caucasian systemic lupus erythematosus (SLE) patients in relation to age at disease onset. Three groups of patients with different ages at disease onset were analysed and compared: group A (30 patients, >or=65 years); group B (62 patients, 50-64 years) and group C (163 patients, <50 years). All patients were regularly followed-up for a mean period of 6.5 years. Female predominance was reduced in groups A and B. Time-lapse between disease onset and diagnosis was longer in group A and B. There were no statistically significant differences in clinical features. The only relevant difference was observed in peripheral nervous system (PNS) involvement, more frequent in group A. Anti-dsDNA and RF were more frequent in group A. Complement levels were reduced more frequently in group C. No differences were observed in disease activity scores, while SLICC/ACR score was higher in group A. In Caucasian SLE patients, age at disease onset is not associated with differences in clinical features apart from a more frequent PNS involvement in elderly patients. In the same group, the organ damage seems to develop more rapidly mostly due to higher susceptibility to jatrogenic side effects.

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Infliximab (chimeric anti-tumour necrosis factor alpha monoclonal antibody) versus placebo in rheumatoid arthritis patients receiving concomitant methotrexate: a randomised phase III trial.

To describe three cases of meningioma observed in a large cohort of 546 patients with systemic lupus erythematosus (SLE) followed at our Department in the last 15 years. We identified three cases of meningioma among 181 patients with SLE who underwent a brain magnetic resonance imaging (MRI) during their disease course (prevalence 1.65%). All three SLE cases were women with a disease onset at 47-, 18- and 42 -years-old, respectively. All patients presented neuropsychiatric (NP) symptoms and had an incidental finding of a meningioma at brain MRI. One patient presented simultaneously a breast cancer. Only one patient had the surgical removal of the mass without improvement of her symptoms while in the other two patients, the removal was not indicated. The association between meningioma and SLE may be a pure coincidence. However, it draws our attention because its detection may represent a confounding factor in the setting of a NPSLE patient.

Among the systemic manifestations of primary Sjögren's syndrome, neurological involvement is still an intriguing and debated issue. Although peripheral nervous system abnormalities are a well documented occurrence with a reported prevalence ranging from 10 to 20%, opinions differ as to the prevalence of CNS disease, with suggestions from 'nonexistent' to 'very common'. The lack of agreement probably reflects the different populations selected, different inclusion criteria and lack of rigorous epidemiological studies. In our experience, CNS involvement was detected in 7 of 87 (8%) unselected consecutive patients observed over a period of 5 years. The spectrum of CNS involvement is wide, including focal, diffuse, neuropsychiatric and spinal cord symptoms, frequently characterised by insidious onset, remitting course and, sometimes, progressive evolution. The diagnostic approach enabling early recognition of this complication relies on careful clinical assessment using history and physical examination combined with neuropsychological testing and instrumental, laboratory and imaging investigations such as magnetic resonance imaging, single photon emission computed tomography, electrophysiological testing and CSF analysis. The clinical picture often shows spontaneous remission, but when overt neurological symptoms occur or become progressive, therapeutic interventions with high dose corticosteroids and cytotoxic agents, such as intravenous cyclophosphamide pulse therapy, may be indicated.

Objective: The aim of this study was to investigate the performance of functional neuro-imaging for describe neurological involvement in Lupus Erithematosus Systemicus. Methods. 20 SLE patients were included into this study (18 females / 2 males). Median age was 40.5 years (range 16 –66 ys), 9 patients with a clear neurological involvement, 7 with aspecific neurological symptoms and 4 were asymptomatics, according to 1999 ACR Classification. All patients were underwent to conventional resonance imaging (RMFLAIR), RM perfusion ( RM-PWI), RM diffusion (RM-DWI) and cerebral SPECT. The RM techniques was performed using a 1 Tesla “Signa-Horizon” Tomograph by General Eletric: The data analysis was performed from two indipendent neuroradiologist and than trough coordinated evalutation after coregistration of acquired volumes. Results: In 11/20 patients (55%) lesions were demonstrated in RM-FLAIR evaluation, more frequent in cases with focal symptoms than in diffuse. RM-PWI was positive in 50% of cases. SPECT analysis was altered in 85 % of patients. In all patients RM-DWI evaluation was negative. 5 of 29 lesional areas (3 patients) showed by SPECT analysis were positive in RM-PWI. None of them was positive in RM-FLAIR study. After coordinated evaluation of RM-FLAIR, SPECT and RM-PWI, 7 findings were considered false positive. 6 Of patients with negative RM-FLAIR were positive in SPECT and 3 in RM-PWI. Only 1 patient was positive in SPECT and RM-PWI. Conclusions: According to the literature, the RM-FLAIR is a very sensitive procedure to describe the lesional charge, especially in patients with focal symptoms. All lesions was considered as stable outcomes due to negativity of RM-DWI analysis. The SPECT is a sensitive technique to individuate cerebral areas of altered perfusion. The coregistration seems to be an helpful method to improve the explanation of uncertain cases. e the sections are prepared for the microscopic analysis of the various histomorphometric parameters

53 patients were included in control group (Group 2). The Lequesne Index was used to determine the severity of the disease. Results All patients in group 1 and 31 patients in group 2 had knee pain and the mean duration of pain was 51.6+77.5 months in group 1 and 23.4+46.5 months in group 2. The mean Body Mass Index (BMI) was 31.7+5.0 in group 1 and 36.7+4.0 in group 2. According to the results of scintigraphic evaluation, healthy controls were separated into two groups. In group 2A patients who had an increase in uptake at least one joint and in group 2B patients who had no uptake. The mean MSS was 4.2 +1.4 in group 1 and 3.3+1.6 in group 2A. Also the mean MRS of the patients in group 1 were 2.9+0.9 and 0.6+0.5, 0.2+0.4 in group 2A and 2B respectively. The mean score of the Lequesne Index was 8,9+3,6 in group 1 patients. There was a positive correlation between age and MSS (p < 0,05) and BMI and MRS (p < 0,05). Also there was a strong correlation between Lequesne OA Severity Index Score the age of the patients in group 1 but no correlation with the MSS. According to the clinical and/or radiological criteria sensitivity of bone scintigraphy was 98.2%, specifity was 57.1%, positive predictive value was 76% and negative predictive value was 96%. Conclusion Our results suggest that 99 m technetium scintigraphy is a better quantifying method in assessing the changes of OA especially in early cases.