Magnetic resonance spectroscopy in the evaluation of central nervous system manifestations of systemic lupus erythematosus.
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Biomedical subjects
Lilian T L Costallat
Publications and source records attributed to Lilian T L Costallat.
Isolated pulmonary hypertension secondary to rheumatoid arthritis.
The authors report a case of a woman with pulmonary hypertension secondary to rheumatoid arthritis, whose treatment with azathioprine resulted in normalization of pulmonary artery pressure and resolution of clinical symptoms. Different etiologies for pulmonary hypertension are discussed and literature review is presented.
Adult↗
Evidence of reversible axonal dysfunction in systemic lupus erythematosus: a proton MRS study.
Our objective was to investigate axonal dysfunction in patients with systemic lupus erythematosus (SLE) using proton magnetic resonance spectroscopy (1H-MRS). We studied prospectively 90 SLE patients (mean age of 32.5 years) and 23 normal volunteers (mean age of 33.8 years). We performed single voxel proton MRS using point resolved spectroscopy sequence over the superior-posterior region of the corpus callosum. We measured signals from N-acetyl compounds [N-acetylaspartate (NAA)] at 2.01 p.p.m., choline-based compounds (Cho) at 3.2 p.p.m. and creatine and phosphocreatine containing compounds (Cr) at 3.0 p.p.m. and determined NAA/Cr ratios. After 12 months, MRI and MRS were repeated in 50 patients and 9 volunteers. Patients were divided according to disease activity (measured by SLE disease activity index) during initial and follow-up MRS. We performed paired t-test and ANOVA with Tukey's post hoc comparisons to evaluate group differences. At study entry, 29 patients had active SLE with involvement of central nervous system (CNS) and 28 patients had active SLE without CNS manifestations. A total of 14 patients had inactive SLE with past CNS presentation, and 19 had inactive SLE without history of CNS involvement. NAA/Cr ratios were significant lower in patients with active SLE, independently of CNS involvement, when compared with patients with inactive SLE (P = 0.005) and controls (P = 0.01). We observed a significant increase in NAA/Cr ratio in 15 patients who had active SLE at initial MRS and inactive SLE at follow-up (P = 0.04). In 10 patients with active SLE both at initial and at follow-up MRS we observed a reduction in NAA/Cr ratio (P = 0.02). By contrast, there was a significant reduction of NAA/Cr ratio in 15 patients who had inactive SLE at initial MRS and active SLE at follow-up (P = 0.001). In 10 patients with inactive SLE both at initial and at follow-up MRS NAA/Cr ratio did not change (P = 0.2). This study shows evidence of axonal dysfunction in patients with active SLE, independently of CNS manifestations that may be reversible, at least in part, during periods of inactivity of disease.
Adult↗
Damage did not independently influence mortality in childhood systemic lupus erythematosus.
OBJECTIVE: To analyze clinical, laboratory and treatment features associated with death in a childhood-onset SLE population. PATIENTS AND METHODS: Patients with childhood-onset SLE followed at the State University of Campinas, Brazil, between 1980 and 2002 were included. Data on clinical and laboratory features of the disease were collected regularly. Logistic regression was used for analyzing association between clinical and laboratory features and death. Kaplan-Meyer tests were used to estimate the survival curves. RESULTS: Of 61 patients identified, six were lost to follow-up during the first year of disease. The mean follow-up period of the remaining 55 patients was 3.25 years (SD=1.2). Mean SLICC/ACR-DI score was 4.9 (SD=3.4). Death occurred in 12 (21.8%) of 55 patients. Direct causes of death were: infection in six (50%), stroke in four (33.3%), and renal insufficiency in two (16.7%). Five patients (41.7%) died during the first 5 years of disease due to infection. Male gender (p=0.004; OR=9.1; 95% CI=7.6-21.0), infection (p=0.001; OR=4.2; 95% CI=1.6-15.2) and nephritis (p=0.02; OR=2.3; 95% CI=1.3-5.2) were independent factors associated with death in the multivariate analysis. The global survival rate adjusted for duration of disease was 93.9% in the first year of disease, 88.9% in the second year, 80.8% in the fifth year and 48.1% in 20 years of follow-up. When comparing survival curves, male gender, the presence of infection during the course of the disease and the presence of nephritis during follow-up had a worse survival. CONCLUSION: Male gender, the presence of infection and nephritis were independent risk factors for death in our Brazilian cohort. Damage did not independently influence survival in this study.
Adolescent↗
Cerebral and corpus callosum atrophy in systemic lupus erythematosus.
OBJECTIVE: To determine cerebral and corpus callosum volumes in patients with systemic lupus erythematosus (SLE), using semiautomatic magnetic resonance imaging (MRI) volumetric measurements, and to determine possible relationships between a reduction in cerebral volume and disease duration, total corticosteroid dose, neuropsychiatric manifestations, and the presence of antiphospholipid antibodies. METHODS: We studied 115 consecutive patients with SLE and 44 healthy volunteers. A complete clinical, laboratory, and neurologic evaluation was performed. MRI scans were obtained through a standardized protocol. Sagittal T1-weighted images were used for semiautomatic volumetric measurements. We compared SLE patients with controls using the 2-sample t-test. Analysis of variance was used to test for differences between groups, followed by Tukey's post hoc test for pairwise comparisons, when necessary. Linear regression was used to analyze the association between cerebral atrophy and disease duration and total corticosteroid dose. RESULTS: Cerebral and corpus callosum volumes were significantly smaller in patients with SLE compared with healthy volunteers (P < 0.001). Reduced cerebral and corpus callosum volumes were related to disease duration (P < 0.001). Patients with a history of central nervous system (CNS) involvement more frequently had a reduction in cerebral and corpus callosum volumes (P < 0.001). Patients with cognitive impairment had significantly reduced corpus callosum and cerebral volumes when compared with SLE patients without cognitive impairment (P = 0.001). Cerebral and corpus callosum volumes were not associated with the total corticosteroid dose or the presence of antiphospholipid antibodies. CONCLUSION: In patients with SLE, a reduction in cerebral and corpus callosum volumes is associated with disease duration, a history of CNS involvement, and cognitive impairment. The total corticosteroid dose and the presence of antiphospholipid antibodies were not associated with more pronounced atrophy.
Adult↗
Adult-onset Still disease in southeast Brazil.
BACKGROUND: Adult-onset Still disease (AOSD) has been described all over the world. Clinical presentations and prognosis have varied in different studies. OBJECTIVE: The objective of this study was to determine the clinical presentation and the evolution of AOSD at a tertiary referral center in southeast Brazil. METHODS: The clinical records of 16 patients were retrospectively studied to determine symptoms at diagnosis, follow up, and the medication prescribed. RESULTS: The mean age at onset was 30.8 years (range, 24-55 years; standard deviation [SD], 9.2 years) with a slight male prevalence (54.2%). All patients presented constitutional symptoms, fever, and skin rash. Liver involvement was observed in all cases, with hepatomegaly in 81.3%, increased liver enzymes in 50.0%, and hypergammaglobulinemia in 68.8%. Cardiac involvement was observed in 12.6%, pleuritis in 6.3%, and renal involvement in 25.0%. All patients presented leukocytosis with a predominance of neutrophils. Elevated ferritin levels were observed in 56.3%, and these levels were normalized after disease remission. Initial treatments included nonsteroidal antiinflammatory drugs and low-dosage corticosteroids in all patients; 43.8% also needed methotrexate. In 25.0% of cases, a monocyclic disease was observed; others had recurrent episodes. After a follow up of 6.9 years (SD, 1.2 years), carpal ankylosis was the main articular sequel, observed in 53.6% of the patients. CONCLUSION: AOSD is rare in southeast Brazil. Although less severe systemic manifestations, like serositis and pneumonitis, were observed, reversible liver involvement was common; the frequency of recurrent disease and carpal ankylosis was higher than in previous studies.
Adult↗
Epileptic seizures in systemic lupus erythematosus.
OBJECTIVE: To evaluate the frequency and risk factors of epileptic seizures in a large cohort of patients with systemic lupus erythematosus (SLE). METHODS: Five hundred nineteen consecutive patients with SLE were studied, with follow-up ranging from 4 to 7.8 years. The type and frequency of risk factors associated with acute and recurrent epileptic seizures in SLE were determined. RESULTS: Sixty (11.6%) patients with epileptic seizures were identified. Epileptic seizures occurred at the onset of SLE symptoms in 19 (31.6%) and after the onset of SLE in 41 of 60 (68.3%) patients. Fifty-three of 60 (88.3%) patients had acute symptomatic epileptic seizures, and 7 of 60 (11.7%) had recurrent epileptic seizures. Variables associated with acute epileptic seizures at SLE onset were stroke (p = 0.0004) and antiphospholipid antibodies (p = 0.0013). Epileptic seizures during follow-up were related to nephritis (p = 0.001), antiphospholipid antibodies (p = 0.005), and epileptic seizures at disease onset (p = 0.00001). All seven patients who presented recurrent epileptic seizures had antiphospholipid syndrome and interictal epileptic abnormalities on EEG. CONCLUSIONS: Epileptic seizures were observed in 11.2% of systemic lupus erythematosus (SLE) patients. Antiphospholipid antibodies and stroke were related to epileptic seizures at SLE disease onset. Patients with renal flares, epileptic seizures at SLE disease onset, and antiphospholipid antibodies were at greater risk for acute symptomatic seizures during follow-up. Recurrence of epileptic seizures occurred in 1.3% of patients and was associated with antiphospholipid syndrome.
Adolescent↗
Cerebral venous thrombosis: influence of risk factors and imaging findings on prognosis.
PURPOSE: To investigate imaging findings, risk factors and outcome in patients with cerebral venous thrombosis (CVT). METHODS: Records of all patients with diagnosis of CVT between 1992 and 2002 were reviewed. Patients with CNS infection and with CVT secondary to invasive procedures were excluded. Inherited and acquired thrombophilia were searched in all patients. RESULTS: Twenty-four patients (18 women, 6 men) with mean age of 29.5 years (range 3-48 years) were identified. Mean follow-up was 44 months (range 11-145 months). The most common symptoms were headache (75%), vomiting (33%) and impairment of consciousness (21%). Probable causes of CVT could be determined in 21 (88%) patients: pregnancy or puerperium in six (25%), oral contraceptive use in four (17%), head trauma in two (8%), mastoiditis in one (4%), nephrotic syndrome in one (4%), systemic disease in three (13%), and inherited thrombotic risk factors in four (17%) patients. CVT associated with pregnancy, puerperium and use of oral contraceptives had a significant better outcome than CVT caused by inherited thrombophilia or systemic disease (OR=14.4; p=0.02). CT scans were abnormal in 15 (62.5%) patients and MRI with gadolinium was abnormal in all. Those with parenchymal involvement had neurological sequelae during follow-up. All were treated with heparin followed by oral anticoagulants, and none had new or worsening of pre-existing intracerebral hemorrhage. CONCLUSION: MRI is superior to conventional CT for diagnosing CVT. Patients with parenchymal lesions, thrombophilia and antiphospholipid syndrome had greater risk to be left with neurological sequelae. Anticoagulant therapy did not predispose to further intracerebral hemorrhage.
Adolescent↗