[show abstract][hide abstract] ABSTRACT: The impact of Behçet's disease on higher cognitive functions is still poorly understood. We proposed (1) to characterize the neuropsychological profile of Behçet's disease patients with (Neuro-BD) and without (BD) neurological manifestations; (2) to identify which clinical, psychopathological, and genetic variables are related to neuropsychological performance; and (3) to explore the association between cognitive functioning and neuroimaging findings in BD patients. Fifteen Neuro-BD and 35 BD patients in the nonactive phase of their illness underwent a neurological examination, performed a comprehensive battery of neuropsychological tests, and answered the hospital anxiety and depression scale. Human leukocyte antigen (HLA)-B*51 genotyping was also performed. Patients' neuropsychological performances were compared to those of healthy demographically matched subjects. Within one month from the testing date, a subset of 20-BD patients underwent a magnetic resonance imaging (MRI) scan. Fifty-three percent of Neuro-BD and 40% of BD patients were impaired at least on one neuropsychological measure (i.e., digit span-forward). Poorer cognitive functioning in Neuro-BD was associated with parenchymal involvement, whereas in BD it was related to presence of white matter changes in the frontal lobes, history of headache complaints, or higher levels of anxiety and depression. Current prednisone intake had a positive impact on neuropsychological performance. Disease duration, time since onset of neurological manifestations, or presence of HLA-B*51 allele had no significant influence. Our results indicate that Behçet's disease may affect cognitive abilities in the absence of overt neurological symptoms. These findings point to an insidious course of neurological involvement.
Annals of the New York Academy of Sciences 09/2009; 1173:217-26. · 4.38 Impact Factor
[show abstract][hide abstract] ABSTRACT: Systemic lupus erythematosus (SLE) is a clinically heterogeneous disease translating the different genetic and environmental factors involved. Polymorphisms at several loci, including the major histocompatibility complex (MHC), have been associated worldwide with SLE, although inconsistencies exist among these studies mainly due to genetic heterogeneity between populations and sample characteristics. The aim of the present study was to investigate in Portuguese SLE the association of HLA-DRB1 alleles with clinical patterns of the disease and severity. Two hundred eighteen Portuguese patients with SLE--42% of whom had kidney involvement--were studied for HLA-DRB1. Clinical and laboratory manifestations were correlated with HLA allele frequencies. HLA-DRB1 * 03 allele frequency was significantly higher in SLE patients--as a whole and as either with or without renal involvement--compared to controls, while HLA-DRB1 * 09 and DRB1 * 13 allele frequencies were decreased. Regarding the relationship with the presence or absence of specific clinical manifestations, it was only found that HLA-DRB1 * 08 allele frequency was increased in patients with neurological involvement. No association with the presence or absence of anti-dsDNA, anti-sm or antiphospholipid antibodies, or antiphospholipid syndrome, was observed. These results were reproducible when analysis was repeated only with patients with more than 5 years of evolution. As in other populations HLA-DRB1 * 03 is a susceptibility allele in Portuguese SLE patients, while HLA-DRB1 * 09 and DRB1 * 13 alleles may be protective alleles, not only for the disease, but for the development of nephritis. No correlations with the different clinical manifestations were found, except with the neurological system.
Annals of the New York Academy of Sciences 09/2009; 1173:575-80. · 4.38 Impact Factor
[show abstract][hide abstract] ABSTRACT: Systemic lupus erythematosus (SLE) is mainly a disease of fertile women and the coexistence of pregnancy is by no means a rare event. How SLE and its treatment affects pregnancy outcome is still a matter of debate. Assessment of the reciprocal clinical impact of SLE and pregnancy was investigated in a cohort study. We reviewed the clinical features, treatment, and outcomes of 43 pregnant SLE patients with 51 pregnancies followed from 1993 to 2007 at a tertiary university hospital. The age of patients was 28.7 +/- 5.4 years and SLE was diagnosed at age of 23.0 +/- 6.1 years. Previous manifestations of SLE included lupus nephritis (14 patients) and secondary antiphospholipid syndrome (11 patients). Thirty-five pregnant patients (69%) were in remission for more than 6 months at the onset of pregnancy. Patients were being treated with low doses of prednisone (29), hydroxychloroquine (20), azathioprine (five), acetylsalicylic acid (51), and low molecular weight heparin (13). Sixteen pregnancy-associated flares were documented, mainly during the second trimester (42%) and also in the following year after delivery (25%). Renal involvement was found in 11 cases (68%). Spontaneous abortion occurred in 6%, 16% had premature deliveries, and 74% were delivered at term. No cases of maternal mortality occurred. No cases of fetal malformation were recorded. There was one intrauterine fetal death and one neonatal death at 24 gestational weeks. Pregnant women with SLE are high risk patients, but we had a 90% success rate in our cohort. A control disease activity strategy to target clinical remission is essential.
[show abstract][hide abstract] ABSTRACT: Systemic Lupus Erythematosus (SLE) is an inflammatory chronic disease characterized by the presence of autoantibodies, immunocomplex production and organ injury. Several alterations of the immune system have been described, namely of CD4 T cells, with particular focus on regulatory subgroup.
Quantify peripheral CD4 T cells in a population of patients with SLE and correlate it with lupus activity, affected organs, therapeutics and infections.
Retrospective study involving all SLE patients seen in the clinical immunology outpatient clinic of the Hospital Geral Santo António, Porto that has done some peripheral blood flow cytometry study.
Twenty-nine patients have been evaluated, 16 were taking glucocorticoids and six immunossupressors. The mean SLEDAI at the study time was nine and the ECLAM was three. Thirty-one percent of the patients had leukopenia, 76% lymphocytopenia and the same number CD4 depletion. Fifty-five percent of the patients had CD4 levels lower than 500/mm3, 31% lower than 200/mm3. All patients with SLEDAI > or = 20 and ECLAM > or = 4 had CD4 counts inferior to 500/mm3 and all patients with inactive disease had CD4 superior to 500/mm3. There have been three opportunistic infections: cryptococcal meningitis, pulmonary aspergilosis, Pneumocystis jirovecii pneumonia, all in patients with CD4 counts lower than 500/mm3.
Decreased CD4 T cells counts have been very common in this study population. There is an inverse relation between CD4 cells counts and disease activity. Opportunistic infections occurred in patients with severe CD4 depletion.