Microstructural Changes in the Corpus Callosum in Systemic Lupus Erythematous.

Central nervous system (CNS) involvement in childhood-onset systemic lupus erythematosus (cSLE) occurs in more than 50% of patients. Structural magnetic resonance imaging (MRI) has identified global cerebral atrophy, as well as the involvement of the corpus callosum and hippocampus, which is associated with cognitive impairment. In this cross-sectional study we included 71 cSLE (mean age 24.7 years (SD 4.6) patients and a disease duration of 11.8 years (SD 4.8) and two control groups: (1) 49 adult-onset SLE (aSLE) patients (mean age of 33.2 (SD 3.7) with a similar disease duration and (2) 58 healthy control patients (mean age of 29.9 years (DP 4.1)) of a similar age. All of the individuals were evaluated on the day of the MRI scan (Phillips 3T scanner). We reviewed medical charts to obtain the clinical and immunological features and treatment history of the SLE patients. Segmentation of the corpus callosum was performed through an automated segmentation method. Patients with cSLE had a similar mid-sagittal area of the corpus callosum in comparison to the aSLE patients. When compared to the control groups, cSLE and aSLE had a significant reduction in the mid-sagittal area in the posterior region of the corpus callosum. We observed significantly lower FA values and significantly higher MD, RD, and AD values in the total area of the corpus callosum and in the parcels B, C, D, and E in cSLE patients when compared to the aSLE patients. Low complement, the presence of anticardiolipin antibodies, and cognitive impairment were associated with microstructural changes. In conclusion, we observed greater microstructural changes in the corpus callosum in adults with cSLE when compared to those with aSLE. Longitudinal studies are necessary to follow these changes, however they may explain the worse cognitive function and disability observed in adults with cSLE when compared to aSLE.

Cognitive Performance in Patients with Systemic Lupus Erythematosus Using the Ped-ANAM.

Computerized batteries have been widely used to investigate cognitive impairment (CI) in patients with SLE. The aim of this study was to evaluate the cognitive performance of patients with SLE in relation to healthy controls using the Pediatric Automated Neuropsychological Assessment Metrics (Ped-ANAM) battery. In addition, we aimed to examine differences in Ped-ANAM scores according to age of disease onset, presence of disease activity, and disease damage. We included 201 consecutive adult-onset (aSLE) and childhood-onset SLE (cSLE) patients who were being followed at the hospital's rheumatology outpatient clinic and 177 healthy controls. We applied the percentage of correct answers on the Ped-ANAM subtests and the Performance Validity Index (PVI) metric and correlated them with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus Erythematosus Damage Index (SDI). Then, we established their relationships with neuropsychiatric systemic lupus erythematosus (NPSLE). We observed CI in a total of 38 (18.9%) SLE patients and 8 (4.5%) healthy controls (p < 0.001). CI was observed in eight (19.5%) cSLE patients and 32 (20%) aSLE patients (p = 0.8175). Individual analysis of the aSLE subtests showed a significant difference in all subtests compared to healthy controls; the greatest differences were in matching to sample (p < 0.001) and memory search ( p < 0.001). In the cSLE group, we observed a difference in the code substitution subtests (p = 0.0065) compared to the healthy controls. In the evaluation of clinical outcomes, disease activity was significantly correlated with CI in cSLE (r = 0.33; p = 0.042) and aSLE (r = 0.40; p = 0.001). We also observed an association between disease activity and neuropsychiatric manifestations (p = 0.0012) in aSLE. In conclusion, we determined that cognitive dysfunction, mainly in memory and attention, was more prevalent in patients with SLE. In both the cSLE and aSLE groups, disease activity was associated with worse cognitive function. This is the first study to use the Ped-ANAM in Brazil. Longitudinal studies are necessary to determine how the Ped-ANAM will perform over time.

Chronic inflammatory demyelinating polyradiculoneuropathy associated with systemic lupus erythematosus.

BACKGROUND: Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is an uncommon subtype of peripheral neuropathy (PN) and especially when associated with systemic lupus erythematosus (SLE). There are few reports characterizing PN-associated to SLE, in particular CIDP. This study reviewed the frequency and profile of SLE-related CIDP in our cohort and in the literature and propose a treatment scheme for CIDP associated with SLE. METHOD: We reviewed our database to identify patients with CIDP and SLE. The literature was also reviewed following the guidelines of PRISMA and using the terms "Polyradiculoneuropathy", "Chronic inflammatory demyelinating polyradiculoneuropathy", "CIDP", "Systemic lupus erythematosus", "SLE", "Autoimmune diseases of the nervous system" until December 2019. Selected articles were published in English. RESULTS: We identified 3 patients with SLE and CIDP in our cohort of 1,349 patients with SLE (0.2%). All patients were female, aged between 30 and 44 years and 2 (66.7%) had active disease in other organs. In the literature, we identified additional 16 patients. A predominance of women with disease activity, specially nephritis and hematological involvement, was observed. Treatment schemes are diverse, including corticosteroids and immunosuppressive drugs. CONCLUSION: Although rare, CIDP has increased frequency in SLE. Women and younger age should rise suspicion of an underlying autoimmune disease. We suggest that CIDP should be included as a possible neuropsychiatric manifestation in SLE.

Familial Aggregation of Childhood- and Adulthood-Onset Systemic Lupus Erythematosus.

OBJECTIVE: To assess the familial occurrence of systemic lupus erythematosus (SLE) in a large Brazilian cohort. METHODS: Consecutive patients with SLE were recruited and stratified according to age at disease onset into childhood-onset SLE or adult-onset SLE. Each patient was personally interviewed regarding the history of SLE across 3 generations (first-, second-, and third-degree relatives). Recurrence rates were analyzed for each degree of relation. RESULTS: We included 392 patients with SLE (112 with childhood-onset SLE and 280 with adult-onset SLE). We identified 2,574 first-degree relatives, 5,490 second-degree relatives, and 6,805 third-degree relatives. In the combined overall SLE cohort, we observed a familial SLE recurrence rate of 19.4 in first-degree relatives, 5.4 in second-degree relatives, and 3.0 in third-degree relatives. Recurrence rates were higher for first- and second-degree relatives of patients with childhood-onset SLE than for first- and second-degree relatives of patients with adult-onset SLE (25.2 versus 18.4 for first-degree, and 8.5 versus 4.5 for second-degree), while in third-degree relatives, recurrence rates were higher in adult-onset SLE than in childhood-onset SLE (P = 2.2 × 10-4 for differences in recurrence proportions between childhood-onset SLE and adult-onset SLE). There were no phenotypic differences in patients from multicase versus single-case families, and there was no sex-skewing observed in the offspring of patients with SLE. CONCLUSION: The greater decline in SLE recurrence rate by generation in childhood-onset SLE versus adult-onset SLE suggests a more polygenic and epistatic inheritance and suggests that adult-onset SLE may be characterized by fewer risk factors that are individually stronger. This finding suggests a higher genetic load in childhood-onset SLE versus adult-onset SLE and a difference in the genetic architecture of the disease based on age at onset.

Disease activity and thromboembolic events in women with systemic lupus erythematosus with and without anti-phospholipid syndrome: users of the 52-mg levonorgestrel-releasing intrauterine system.

PURPOSE: The disease status and thromboembolic events in women with systemic lupus erythematosus (SLE), with and without anti-phospholipid syndrome (APS), were evaluated before and after placement of the 52-mg levonorgestrel-releasing intrauterine system (LNG-IUS). METHODS: A retrospective cohort study, with review of medical records of SLE women, who received an LNG-IUS placement between January 2007 and December 2016, carried out at the University of Campinas Medical School, Brazil. The outcomes included the disease activity (SLEDAI-2K) and damage index scores (SLICC/ACR-DI) presented for each year of device use, as well as venous/arterial thrombotic events, insertion up to a median of 5 years. The author's used χ2, Fisher's exact and the Mann-Whitney tests for analysis and generalized estimating equations for score comparison. RESULTS: The study evaluated 46 women with SLE, 18 with and 28 without APS; the mean age (± standard deviation [SD]) was 31.8 (SD ± 8.3) years old. The length of follow-up after LNG-IUS placement was 5.6 (SD ± 2.7) and 4.1 (SD ± 2.3) years for the groups with and without APS, respectively. Comparison of the groups found that the SLEDAI and SLICC mean scores were low for both at baseline, without variations through the follow-up. After LNG-IUS placement, two women presented three thrombotic arterial events, and one of them died from causes unrelated to LNG-IUS use. CONCLUSIONS: Our results, although restricted, provide information to policymakers and health professionals that the use of a 52 mg LNG-IUS over a 5-year median did not increase disease activity or damage index scores among women with SLE, with and without APS.

Olfactory function in systemic lupus erythematosus and systemic sclerosis. A longitudinal study and review of the literature.

BACKGROUND/PURPOSE: To evaluate olfactory function in systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and healthy controls over a 2-year period, and to determine the association of olfactory dysfunction with age, disease activity, disease damage, treatment, anxiety and depression symptoms and limbic structures volumes. METHODS: Consecutive SLE and SSc patients were enrolled in this study. Clinical, laboratory disease activity and damage were assessed according to diseases specific guidelines. Olfactory functions were evaluated using the Sniffin' Sticks test (TDI). Volumetric magnetic resonance imaging (MRI) was obtained in a 3T Phillips scanner. Amygdalae and hippocampi volumes were analyzed using FreeSurfer® software. RESULTS: We included 143 SLE, 57 SSc and 166 healthy volunteers. Olfactory dysfunction was observed in 78 (54.5%) SLE, 35 (59.3%) SSc patients and in 24 (14.45%) controls (p<0.001) at study entry. SLE and SSc patients had significantly lower mean in all three phases (TDI) of the olfactory assessment when compared with healthy volunteers. In SLE, the presence of olfactory dysfunction was associated with older age, disease activity, higher anxiety and depression symptoms score, smaller left hippocampus volume, smaller left and right amygdalae volume and the presence of anti-ribosomal P (anti-P) antibodies. In SSc the presence of olfactory impairment was associated with older age, disease activity, smaller left and right hippocampi volumes and smaller right amygdala volume. Olfactory function was repeated after a 2-year period in 90 SLE, 35 SSc and 62 controls and was stable in all three groups. CONCLUSION: Both SLE and SSc patients with longstanding disease had significant reduction in all stages of TDI that maintained stable over a 2-year period. Olfactory dysfunction was associated with age, inflammation and hippocampi and amygdalae volumes. In SLE, additional association with anti-P, anxiety and depression symptoms was observed.

Low-Density Lipoprotein Cholesterol Is Associated With Asymptomatic Sensorineural Hearing Loss in Patients With Systemic Lupus Erythematosus.

OBJECTIVE: The aims of this study were to determine the frequency of asymptomatic sensorineural hearing loss (SNHL) in systemic lupus erythematosus (SLE) and to determine the association between SNHL and demographic, clinical, and laboratory features and cardiovascular risk factors. METHODS: We conducted a cross-sectional study including consecutive female SLE patients. We performed audiometry and clinical and laboratory evaluation and determined cardiovascular risk factors in all patients. Statistical analysis included principal component analysis and logistic regression. RESULTS: Eighty-nine women were included with mean age of 38.98 (SD, 7.77) years and mean disease duration of 10.29 (SD, 9.19) years. Asymptomatic SNHL was observed in 14 patients (16%). In logistic regression model, only low-density lipoprotein levels (z = 2.64; P = 0.008) were associated with SNHL. CONCLUSIONS: We observed asymptomatic SNHL in 16% of SLE and an association with low-density lipoprotein levels suggesting atherosclerosis as a mechanism. Follow-up is needed to determine clinical implications.

Superoxide release in juvenile systemic lupus erythematosus.

The objective of this study was to analyze the un-stimulated and stimulated release of superoxide anion (O(2) (-)) by granulocytes and monocytes in juvenile systemic lupus erythematosus (jSLE). The un-stimulated and phorbol myristate acetate (PMA, 30 nM)-induced O(2) (-)by granulocytes and monocytes were determined in six different times of incubation in patients with 23 jSLE and 28 controls. The analysis compared the jSLE group, which was classified into two subgroups by SLEDAI in one inactive subgroup (score <3) (n = 13 patients) and one active subgroup (score ≥3) (n = 10 patients) to the same control group. At time of blood withdrawal, 13 (56.52%) had inactive and 10 (43.47%) patients had active SLE. jSLE patients' granulocytes and monocytes had always a lower un-stimulated O(2) (-) production when compared to controls. Stimulated granulocytes had an increased O(2) (-) production at baseline followed by a significant lower production at 60 min in jSLE when compared to controls. Stimulated monocytes had a similar O(2) (-) production among patients with jSLE and controls. The results suggest a defect in phagocytic function in jSLE. The significant higher release of O(2) (-) in the assays of the stimulated granulocytes, in the initial instances, the so-called respiratory burst, could be attributed to the inflammatory state of phagocytes.

Neuropsychiatric manifestations in systemic lupus erythematosus: epidemiology, pathophysiology and management.

Systemic lupus erythematosus (SLE) is a relapsing-remitting autoimmune disease with CNS involvement occurring in up to 75% of patients. However, the frequency of neuropsychiatric manifestations in SLE studies varies widely, depending on the type of manifestations included and the method used for evaluation. CNS involvement may be considered primary if directly related to SLE activity in the CNS or secondary when related to treatment, infections, metabolic abnormalities or other systemic manifestations such as uraemia and hypertension. The pathogenesis of neuropsychiatric SLE is as yet unknown, though numerous autoantibodies and cytokines have been suggested as possible mediators. However, independent of the aetiology of the insult, the final common pathway in neuropsychiatric SLE is the involvement of the cerebral microvasculature. The diagnosis of primary CNS involvement by SLE is often difficult, as both focal and diffuse manifestations may occur and there is no gold standard for diagnosis. A high index of clinical suspicion, in addition to laboratory and neuroimaging findings may support the diagnosis. Treatment is mostly empirical, although one randomized controlled trial has shown that cyclophosphamide in addition to methylprednisolone is superior to methylprednisolone alone in severe neuropsychiatric SLE.

Prevalence of thyroid dysfunction in systemic lupus erythematosus.

OBJECTIVE: To report the prevalence and clinical relevance of autoimmune thyroid disease and thyroid antibodies in 524 patients with SLE. METHODS: The medical charts of SLE patients followed in our rheumatology unit were reviewed to determine the prevalence and clinical associations of autoimmune thyroid disease. These findings were compared with the prevalence of autoimmune thyroid disease in 50 female adults. Chi(2) tests and Fisher exact tests were used in the comparison of the groups for the categorical variables and Mann-Whitney U test for the continuous variables. Spearman rank correlation was used to identify an association between thyroid symptoms and disease activity. RESULTS: Symptomatic autoimmune thyroid disease was observed in 32 of 524 (6.1%) SLE patients and in 1 of 50 controls (P > 0.05), predominantly hypothyroidism (28 SLE patients vs. in 1 control). Subclinical thyroid disease was identified in 60 (11.5%) and positive thyroid autoantibodies in the absence of thyroid disease in 89 of 524 (17%) SLE patients. Thyroid autoantibodies preceded the occurrence of clinical autoimmune thyroid disease in 70% of SLE patients. Sjögren syndrome (P = 0.001) and positive rheumatoid factor (P = 0.04) were more frequently observed in SLE patients with autoimmune thyroid disease when compared with SLE patients without autoimmune thyroid disease. Disease activity of the SLE was correlated with the presence of symptoms of hyperthyroidism (r = 0.4; P = 0.004). CONCLUSION: Our patients with SLE had a high prevalence of symptomatic and significantly more subclinical hypothyroidism and positive thyroid autoantibodies. Thyroid autoantibodies may precede the appearance of clinical autoimmune disease. Sjögren syndrome and positive rheumatoid factors were more frequently observed in SLE patients with autoimmune thyroid disease. We believe that, since symptoms of SLE and thyroid disease can be similar, that SLE patients should routinely been investigated for autoimmune thyroid disease.

Quantitative magnetic resonance imaging analyses and clinical significance of hyperintense white matter lesions in systemic lupus erythematosus patients.

OBJECTIVE: To analyze the clinical significance of hyperintense white matter (WM) lesions in both symptomatic and asymptomatic systemic lupus erythematosus (SLE) patients. METHODS: We studied 120 consecutive SLE patients and 44 healthy volunteers. Fluid attenuated inversion recovery and T2-weighted magnetic resonance images (MRI) were used for visual and semiautomatic volumetric measurements. RESULTS: At baseline, 61 MRI were normal and 59 had hyperintense WM lesions. Mean volumes of WM lesions were 96.14 (SD = 85.14) mm(3) in T2 weighted and 197.2 (161.13) mm(3) in FLAIR images. The volume of WM lesions was associated with age (r = 0.45; p = 0.01), total corticosteroid dose (r = 0.53; p = 0.001), and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index scores (r = 0.55; p = 0.002). After a median follow-up time of 24 months (SD = 2.3; range = 12-28 months), 20 patients had still normal MRIs, 30 patients had stable MRI findings, and 30 had new WM lesions. Predictors for new or increased WM lesions were past central nervous system manifestations (p = 0.001; OR = 12.2; 95% CI = 3.5-21.2), antiphospholipid antibodies (p = 0.003; OR = 6.9; 95% CI = 2.1-15.3); Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index scores (p = 0.002; OR = 7.2; 95% CI = 1.4-17.8) and higher dose of total corticosteroid dose (p = 0.01; OR = 2.4; 95% CI = 1.4-6.7). CONCLUSION: Small hyperintense WM lesions in SLE are associated with central nervous system symptoms and antiphospholipid antibodies, and progress over time in patients with more severe SLE. Therefore, in the context of SLE, these lesions are likely consequences of central nervous system damage and not mere incidental finding.

Systemic lupus erythematosus in patients with sickle cell disease.

Sickle cell disease (SCD) is a prevalent genetic disorder that includes sickle cell anemia (hemoglobin SS), hemoglobin SC, and hemoglobin Sb-thalassemia. Patients with SCD present with a defective activation of the alternate pathway of the complement system that increases the risk of capsulate bacteria infection and failure to eliminate antigens, predisposing these patients to autoimmune diseases. The authors describe three patients with SCD that developed systemic lupus erythematosus (SLE). In all patients, SLE diagnosis was delayed because symptoms were initially attributable to SCD. Physicians should be alerted to the possible development of SLE in patients with SCD to not delay the diagnosis and start appropriate treatment.

Frequency of HLA-B27 and its alleles in patients with Reiter syndrome: comparison with the frequency in other spondyloarthropathies and a healthy control population.

This retrospective study analyzed the HLA-B 27 alleles in a group of 20 consecutive patients with the diagnosis of Reiter syndrome (RS) followed in a tertiary referral university hospital in Brazil, during the period 1990-2006, and compared the data with that observed in other patients with spondyloarthropathies followed at the same institution. Eight cases were associated to gastrointestinal infection, eight cases to previous urethritis, and four cases presented no established preceding infection. HLA-B 27 alleles were typed by polymerase chain reaction-amplified DNA hybridized with sequence-specific oligonucleotide probes (HLA-B 2,701 to HLA-B 2,721). They were compared to a group of 108 patients with ankylosing spondylitis (AS), 40 with undifferentiated spondyloarthropathy (uSpA) and 111 healthy controls. Among the 20 patients, 17 were HLA-B 27 positive (85%). Two HLA-B 27 alleles were observed: HLA-B 2,705 (65%) and HLA-B 2,702 (35%). In the other spondyloarthropathies, the observed alleles were HLA-B 2,705 (90% in AS and 92.5% in uSpA), HLA-B 2,702 (8% in AS and 5% in uSpA), HLA-B 2,704 (1% in AS and 2.5% in uSpA) and HLA-B 2,713 (1% in AS). Among the 111 healthy controls, 80% presented HLA-B 2,705, followed by HLA-B 2,702 in 10%, HLA-B 2,703 in 6%, HLA-B 2,707 in 3% and HLA-B 2,713 in 1%. Concluding, in the HLA-B 27 positive patients with RS in this study there was predominance of HLA-B 2,705 allele, in a lower frequency than that observed in patients with other spondyloarthropathies and healthy controls.

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.

Acute dermatomyositis with subcutaneous generalized edema.

The authors report a 40-year-old Caucasian man with relapsing muscle and skin involvement of dermatomyositis treated with high-dose corticosteroids, taken orally, and methotrexate and human gamma globulin, both administered intravenously. After 4 months of aggressive treatment, he presented with generalized edema, considered secondary to dermatomyositis. Aggressive immunosuppression did not stop disease progression. The literature concerning anasarca due to inflammatory myopathies is revised.

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.

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.

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.