Objective-To investigate prospectively abnormalities of brain glucose utilisation in relation to major or minor neuropsychiatric symptoms in systemic lupus erythematosus (SLE). Methods-Positron emission tomography (PET) using F-18-labelled fluorodeoxyglucose was performed in 28 patients with SLE. Patients were classified as having severe neuropsychiatric manifestations (seizures, focal neurological deficits, acute confusional states, mood disorders) (n=12), or mild neuropsychiatric manifestations (headache, reactive depression, cognitive dysfunction, anxiety disorders) (n=11) and five patients without signs of central nervous system (CNS) involvement. Ten clinically and neurologically healthy volunteers served as controls. In 26 patients magnetic resonance imaging (MRI) was performed and autoantibodies against CNS tissue, ribosomal P protein and cardiolipin were measured. In 14 patients follow up PET scans were performed after a mean (SD) period of 11.6 (9.5) months. Results-PET scans showed hypometabolism in at least one brain region in all patients with severe or mild CNS symptoms (100%) as compared with patients without cerebral symptoms (40%) (p<0.0025). Parieto-occipital regions were most commonly aVected (96%), followed by parietal regions (32%). In contrast, MRI images were abnormal in only 11 of 22 patients (50%) with neuropsychiatric symptoms and in one of four patients (25%) without symptoms. In 12 of 14 patients examined in follow up PET scans persistence, improvement or worsening of cerebral symptoms were associated with unchanged, decreased or increased brain hypometabolism, respectively. No significant correlation was found between PET or MRI findings and autoantibody profiles. Conclusions-PET imaging represents a sensitive tool to detect manifest or subclinical CNS involvement in SLE and PET findings correlate well with the clinical course of disease.
In both northern and southern Germany, GCA was significantly more prevalent in urban than in rural populations, especially among people aged > or =50 yr and in women. It remains unclear whether this disparity was due to underdiagnosis of GCA in the rural regions associated with differences in the German health-care system in cities vs rural areas. Further studies must examine the role of (chronic) exposure to the environmental factors characteristic of cities.
Several lines of evidence argue in favour of an involvement of T cells in the pathogenesis of Wegener's granulomatosis (WG). These include the presence of highly specific IgG autoantibodies to proteinase 3, perivascular T-cell infiltrates and elevated amounts of soluble interleukin-2 (IL-2) receptors in patient's serum. In order to further address this question we evaluated by double immunofluorescence and flow cytometry the expression of several cell surface molecules associated with T-cell activation. As compared to healthy controls (n = 15), the CD4+ subset was significantly diminished, while the percentage of CD8+ T cells was elevated in WG patients (n = 24). Within the CD4+ T-cell subset we found a highly significant increase in activation/memory markers (CD25, CD29, HLA-DR). Within the CD8+ T-cell subset the expression of CD11b, CD29 and CD57 was significantly elevated, while the expression of VD28 was reduced. The use of 10 V beta-, 1 V alpha- and 1 V gamma-specific monoclonal reagents failed to reveal any significant bias in the peripheral T-cell receptor V-gene repertoire of WG patients. There was also no correlation between T-cell activation markers and laboratory parameters [C-reactive protein (CRP), ESR], disease duration or therapy. A significant correlation was found only for the degree of organ involvement and the increase in CD4+ T cells coexpressing HLA-DR, as well as the increase in CD57 expression on CD8+ T cells. In conclusion, both CD4+ and CD8+ T-cell subsets were activated in WG. Cytotoxic CD8+CD57+CD11b+CD28- T cells may directly contribute to damage of vascular endothelium.
First results from a population-based vasculitis register serving nearly 5,000,000 inhabitants in north and south Germany revealed no regional differences in the incidence of all PSVs between north and south. The incidence rates of ANCA-associated PSVs, such as WG and MPA, were lower than those in the UK and Norway but higher than that in Spain.
Objectives. To evaluate the effects of Wegener's granulomatosis (WG) on employment status, work disability, and need for medical care of 60 consecutive WG patients aged <40 years at diagnosis. Methods. Sixty WG patients (26 male, 34 female) with a median age of 36 years (range 17-48 years) and a median duration of disease of 39 months (range 0 -228 months) completed self-administered questionnaires on hospitalization, medical care, and employment status plus the Medical Outcomes Study-Short Form-36 (SF-36) estimating their healthrelated quality of life. Results. Thirty-two of the 60 patients reported full-or part-time employment more than 3 years after diagnosis. Only 14 of the 51 patients employed at diagnosis (27%) were currently receiving a permanent work disability pension due to WG. Two additional patients had lost work because of WG. Women who were employed at diagnosis had a nearly 3-fold higher risk of losing their jobs compared with men (P ؍ 0.0006). There were no differences with regard to age at diagnosis, disease duration, disease severity, or education level between employed and unemployed patients. Employed patients had missed a median of 14 workdays (range 0 -18 days) due to WG within the past 12 months. More than half of all patients (33 of 60) had been hospitalized during the previous 12 months because of WG. Ninety-three percent of all patients had visited their physician once or more per month, more than half of them at least once per week, regardless of employment status, severity of disease, or type of current medication. Unemployed WG patients experienced significant reductions in social and physical function and in their perceived degree of general health as assessed by the SF-36. Conclusions. Twenty-seven percent of WG patients younger than age 40 who were employed at diagnosis received permanent work disability within a disease duration of 39 months. Unemployment is followed by a considerable reduction in disease-related quality of life compared with employed patients, independent of severity and extent of disease. Furthermore, because patients were followed closely by an interdisciplinary team, a high rate of hospitalization and frequent visits to physicians resulted.
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