In-person versus virtual administration of the American College of Rheumatology gold standard cognitive battery in systemic lupus erythematosus: Are they interchangeable?

OBJECTIVE: During the COVID-19 pandemic, many research studies were adapted, including our longitudinal study examining cognitive impairment (CI) in systemic lupus erythematosus (SLE). Cognitive testing was switched from in-person to virtual. This analysis aimed to determine if the administration method (in-person vs. virtual) of the ACR-neuropsychological battery (ACR-NB) affected participant cognitive performance and classification. METHODS: Data from our multi-visit, SLE CI study included demographic, clinical, and psychiatric characteristics, and the modified ACR-NB. Three analyses were undertaken for cognitive performance: (1) all visits, (2) non-CI group visits only and (3) intra-individual comparisons. A retrospective preferences questionnaire was given to participants who completed the ACR-NB both in-person and virtually. RESULTS: We analysed 328 SLE participants who had 801 visits (696 in-person and 105 virtual). Demographic, clinical, and psychiatric characteristics were comparable except for ethnicity, anxiety and disease-related damage. Across all three comparisons, six tests were consistently statistically significantly different. CI classification changed in 11/71 (15%) participants. 45% of participants preferred the virtual administration method and 33% preferred in-person. CONCLUSIONS: Of the 19 tests in the ACR-NB, we identified one or more problems with eight (42%) tests when moving from in-person to virtual administration. As the use of virtual cognitive testing will likely increase, these issues need to be addressed - potentially by validating a virtual version of the ACR-NB. Until then, caution must be taken when directly comparing virtual to in-person test results. If future studies use a mixed administration approach, this should be accounted for during analysis.

Magnetic resonance imaging assessment of juvenile idiopathic arthritis using OMERACT and EuroTMjoint classifications.

This retrospective case-control study compared inflammatory and structural damage in the temporomandibular joint of patients with juvenile idiopathic arthritis (JIA) and its subtypes and healthy patients using the Outcome Measures in Rheumatology Clinical Trials (OMERACT) and EuroTMjoint classifications. Correlations between the scores of the two classifications and time of diagnosis were evaluated. Twenty-nine JIA patients and 48 age-matched healthy participants were examined. TMJ images on each side were considered individually. Oligoarticular and polyarticular subtypes were present in 44.8% and 55.2% of patients, respectively. The JIA group presented a higher frequency and more severe signs of inflammatory and structural changes (P < 0.05), except for effusion (P = 0.83). The polyarticular subtype showed a higher change intensity. The time of JIA diagnosis was not correlated with inflammatory and structural changes. Positive correlations between inflammation and bone deformity scores were observed for the EuroTMjoint classification (r = 0.462, P < 0.001; low correlation) and OMERACT classification (r = 0.737, P < 0.001; high correlation). Positive correlations between the OMERACT and EuroTMjoint classifications were found for inflammation score (r = 0.907, P < 0.001; very high correlation) and bone deformity score (r = 0.854, P < 0.001; high correlation). Both classifications showed a higher frequency and intensity of inflammation and bone deformity in JIA patients. The results of this study suggest that the appropriate management of inflammation may reduce the potential for structural damage to the TMJ.

Cutaneous epithelioid haemangiomas show somatic mutations in the mitogen-activated protein kinase pathway.

BACKGROUND: Epithelioid haemangioma (EH) arising from the skin is a benign vascular tumour with marked inflammatory cell infiltration, which exhibits a high tendency to persist and frequently recurs after resection. So far, the underlying pathogenesis is largely elusive. OBJECTIVES: To identify genetic alterations by next-generation sequencing and/or droplet digital polymerase chain reaction (ddPCR) in cutaneous EH. METHODS: DNA and RNA from an EH lesion of an index patient were subjected to whole-genome and RNA sequencing. Multiplex PCR-based panel sequencing of genomic DNA isolated from archival formalin-fixed paraffin-embedded tissue of 18 patients with cutaneous EH was performed. ddPCR was used to confirm mutations. RESULTS: We identified somatic mutations in genes of the mitogen-activated protein kinase (MAPK) pathway (MAP2K1 and KRAS) in cutaneous EH biopsies. By ddPCR we could confirm the recurrent presence of activating, low-frequency mutations affecting MAP2K1. In total, nine out of 18 patients analysed showed activating MAPK pathway mutations, which were mutually exclusive. Comparative analysis of tissue areas enriched for lymphatic infiltrate or aberrant endothelial cells, respectively, revealed an association of these mutations with the presence of endothelial cells. CONCLUSIONS: Taken together, our data suggest that EH shows somatic mutations in genes of the MAPK pathway which might contribute to the formation of this benign tumour.

Atlas of MRI findings of sacroiliitis in pediatric sacroiliac joints to accompany the updated preliminary OMERACT pediatric JAMRIS (Juvenile Idiopathic Arthritis MRI Score) scoring system: Part I: Active lesions.

Magnetic resonance imaging (MRI) is an increasingly important tool for identifying involvement of the sacroiliac joints (SIJ) in juvenile idiopathic arthritis (JIA). The key feature for diagnosing active sacroiliitis is bone marrow edema (BME), but other features of active arthritis such as joint space inflammation, inflammation in an erosion cavity, capsulitis and enthesitis can be seen as well. Structural changes may also be seen. Systematic MRI assessment of inflammation and structural damage may aid in monitoring the disease course, choice of therapeutics and evaluating treatment response. In this pictorial essay, we illustrate normal MRI findings and growth-related changes of the SIJ in the pediatric population, as well as the different MRI features of SIJ inflammation. This atlas demonstrates fundamental MRI disease features of active inflammation in a format that can serve as a reference for assessing SIJ arthritis according to the updated preliminary JAMRIS (Juvenile Idiopathic Arthritis MRI Score) scoring system proposed by the MRI in JIA working group of Outcome Measures in Rheumatology and Clinical Trials (OMERACT). The atlas is intended to be read in conjunction with its companion Part 2, Structural Lesions.

Atlas of MRI findings of sacroiliitis in pediatric sacroiliac joints to accompany the updated preliminary OMERACT pediatric JAMRIS (Juvenile Idiopathic Arthritis MRI Score) scoring system: Part II: Structural damage lesions.

Magnetic resonance imaging (MRI) is the imaging modality of choice for identifying sacroiliitis in juvenile idiopathic arthritis (JIA). Besides active lesions of sacroiliitis, of which bone marrow edema (BME) is the key feature, structural damage lesions can also be detected. Structural changes include erosion, sclerosis, fat lesion, backfill and ankylosis, and are more common at later stages. Systematic MRI assessment of inflammation and structural damage may aid in monitoring the course of the disease and evaluating treatment options. In this pictorial essay, we illustrate normal MRI findings and growth-related changes of the SIJ in the pediatric population, as well as the different MRI features of structural damage of sacroiliitis. This atlas can serve as a reference for assessing structural lesions of SIJ arthritis according to the updated preliminary JAMRIS (Juvenile Idiopathic Arthritis MRI Score) scoring system proposed by the MRI in JIA working group of Outcome Measures in Rheumatology and Clinical Trials (OMERACT). The atlas is intended to be read in conjunction with its companion Part 1, Active Lesions.

Characteristics of 1555 childhood-onset lupus in three groups based on distinct time intervals to disease diagnosis: a Brazilian multicenter study.

Objective The objective of this study was to compare demographic data, clinical/laboratorial features and disease activity at diagnosis in three different groups with distinct time intervals between onset of signs/symptoms and disease diagnosis. Methods A multicenter study was performed in 1555 childhood-onset systemic lupus erythematosus (American College of Rheumatology criteria) patients from 27 pediatric rheumatology services. Patients were divided into three childhood-onset systemic lupus erythematosus groups: A: short time interval to diagnosis (<1 month); B: intermediate time interval (≥1 and <3 months); and C: long time interval (≥3 months). An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2 K were evaluated. Results The number of patients in each group was: A = 60 (4%); B = 522 (33.5%); and C = 973 (62.5%). The median age at diagnosis (11.1 (4.2-17) vs. 12 (1.9-17.7) vs. 12.5 (3-18) years, P = 0.025) was significantly lower in group A compared with groups B and C. The median number of diagnostic criteria according to SLICC (7 (4-12) vs. 6 (4-13) vs. 6 (4-12), P < 0.0001) and SLEDAI-2 K (18 (6-57) vs. 16 (2-63) vs. 13 (1-49), P < 0.0001) were significantly higher in group A than the other two groups. The frequency of oral ulcers in the palate (25% vs. 15% vs. 11%, P = 0.003), pleuritis (25% vs. 24% vs. 14%, P < 0.0001), nephritis (52% vs. 47% vs. 40%, P = 0.009), neuropsychiatric manifestations (22% vs. 13% vs. 10%, P = 0.008), thrombocytopenia (32% vs. 18% vs. 19%, P = 0.037), leucopenia/lymphopenia (65% vs. 46% vs. 40%, P < 0.0001) and anti-dsDNA antibodies (79% vs. 66% vs. 61%, P = 0.01) were significantly higher in group A compared with the other groups. In contrast, group C had a less severe disease characterized by higher frequencies of synovitis (61% vs. 66% vs. 71%, P = 0.032) and lower frequencies of serositis (37% vs. 33% vs. 25%, P = 0.002), proteinuria >500 mg/day (48% vs. 45% vs. 36%, P = 0.002) and low complement levels (81% vs. 81% vs. 71%, P < 0.0001) compared with groups A or B. Conclusions Our large Brazilian multicenter study demonstrated that for most childhood-onset systemic lupus erythematosus patients, diagnosis is delayed probably due to mild disease onset. Conversely, the minority has a very short time interval to diagnosis and a presentation with a more severe and active multisystemic condition.

Depression in systemic lupus erythematosus: gender differences in the performance of the Beck Depression Inventory (BDI), Center for Epidemiologic Studies Depression Scale (CES-D), and Hospital Anxiety and Depression Scale (HADS).

The prevalence of depressive symptoms in patients with systemic lupus erythematosus (SLE) varies widely between different cohorts (17-75%), primarily due to factors such as the heterogeneity of the samples and the instruments used to detect depressive symptoms. Most of these instruments are self-administered questionnaires that have different characteristics and approaches to depressive symptoms. This study aimed to evaluate gender differences in the performance of three questionnaires used to assess depressive symptoms in patients with SLE: the Beck Depression Inventory (BDI), Center for Epidemiologic Studies Depression Scale (CES-D), and Hospital Anxiety and Depression Scale (HADS). This study included 54 male and 54 female SLE patients. Depressive symptoms were assessed using BDI (cutoffs 13 and 15), CES-D and HADS. The gold standard method used was the diagnostic criteria of the fifth edition of the Diagnostic and Statistical Manual of Mental Disorders. Regarding the performance of the BDI questionnaire, no significant differences in sensitivity or specificity were found between the genders. The specificity of the CES-D questionnaire was significantly greater for the male group (83% vs. 62.5%, p = 0.0309), and its sensitivity was non-significantly higher for the female group (92.9% for women and 71.4% for men; p = 0.2474). Regarding the performance of the HADS, we found similar sensitivities between the genders (71.4%) but a higher specificity among the men (95.7% in men and 82.5% in women, p = 0.0741). In conclusion, our results suggest the presence of gender differences in the performance of the questionnaires in SLE patients. The BDI had the most similar performances between the male and female groups. In contrast, the CES-D and HADS-D showed considerable variation in performances between men and women with SLE.

Computational methods for corpus callosum segmentation on MRI: A systematic literature review.

BACKGROUND AND OBJECTIVE: The corpus callosum (CC) is the largest white matter structure in the brain and has a significant role in central nervous system diseases. Its volume correlates with the severity and/or extent of neurodegenerative disease. Even though the CC's role has been extensively studied over the last decades, and different algorithms and methods have been published regarding CC segmentation and parcellation, no reviews or surveys covering such developments have been reported so far. To bridge this gap, this paper presents a systematic literature review of computational methods focusing on CC segmentation and parcellation acquired on magnetic resonance imaging. METHODS: IEEExplore, PubMed, EBSCO Host, and Scopus database were searched with the following search terms: ((Segmentation OR Parcellation) AND (Corpus Callosum) AND (DTI OR MRI OR Diffusion Tensor Imag* OR Diffusion Tractography OR Magnetic Resonance Imag*)), resulting in 802 publications. Two reviewers independently evaluated all articles and 36 studies were selected through the systematic literature review process. RESULTS: This work reviewed four main segmentation methods groups: model-based, region-based, thresholding, and machine learning; 32 different validity metrics were reported. Even though model-based techniques are the most recurrently used for the segmentation task (13 articles), machine learning approaches achieved better outcomes of 95% when analyzing mean values for segmentation and classification metrics results. Moreover, CC segmentation is better established in T1-weighted images, having more methods implemented and also being tested in larger datasets, compared with diffusion tensor images. CONCLUSIONS: The analyzed computational methods used to perform CC segmentation on magnetic resonance imaging have not yet overcome all presented challenges owing to metrics variability and lack of traceable materials.

Magnetic resonance imaging in neuropsychiatric systemic lupus erythematosus: current state of the art and novel approaches.

Systemic lupus erythematosus is a chronic, inflammatory, immune-mediated disease affecting 0.1% of the general population. Neuropsychiatric manifestations in systemic lupus erythematosus have been more frequently recognized and reported in recent years, occurring in up to 75% of patients during the disease course. Magnetic resonance imaging is known to be a useful tool for the detection of structural brain abnormalities in neuropsychiatric systemic lupus erythematosus patients because of the excellent soft-tissue contrast observed with MRI and the ability to acquire multiplanar images. In addition to conventional magnetic resonance imaging techniques to evaluate the presence of atrophy and white matter lesions, several different magnetic resonance imaging techniques have been used to identify microstructural or functional abnormalities. This review will highlight different magnetic resonance imaging techniques, including the advanced magnetic resonance imaging methods used to determine central nervous system involvement in systemic lupus erythematosus.

S100ß is associated with cognitive impairment in childhood-onset systemic lupus erythematosus patients.

Objective To investigate serologic S100ß protein levels in childhood-onset SLE patients (cSLE) and to elucidate their association with disease activity and neuropsychiatric (NP) manifestations. Methods We included 71 cSLE patients (67 females; median age 18 years; range 9-37 and 53 (47 females; median age of 20 years; range 6-29) age and sex matched healthy controls. Neurological manifestations were analysed according to the American College of Rheumatology (ACR) criteria. Cognitive evaluation was performed in all participants using Wechsler Intelligence Scale for Children (WISC-III) and Wechsler Adult Intelligence Scale (WAIS), according to age, and validated in Portuguese. SLE patients were further assessed for clinical and laboratory SLE manifestations, disease activity (SLE Disease Activity Index (SLEDAI)), damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)) and current drug exposures. Sera S100ß protein levels were measured by enzyme-linked immunosorbent assay using commercial kits. Results The median S100ß protein level was 116.55 pg/mL (range 1.53-468.50) in cSLE and 54.98 pg/mL (range 0.69-181.00) in healthy controls ( p < 0.001). An association was observed between S100ß protein and NP manifestations ( p = 0.03). The S100ß protein levels was associated with cognitive impairment in cSLE patients ( p = 0.006). Conclusions S100ß protein levels are increased in cSLE with cognitive impairment. S100ß may be considered a potential biomarker that underlies central nervous system (CNS) dysfunction, especially cognitive impairment.

Anti-ribosomal P antibody: a multicenter study in childhood-onset systemic lupus erythematosus patients.

Objectives Anti-ribosomal P protein (anti-P) autoantibodies are highly specific for systemic lupus erythematosus (SLE). However, the evaluation of this autoantibody in childhood-onset SLE (cSLE) populations has been limited to a few small series, hampering the interpretation of the clinical and laboratorial associations. Therefore, the objective of this multicenter cohort study was to evaluate demographic, clinical/laboratorial features, and disease damage score in cSLE patients with and without the presence of anti-P antibody. Methods This was a retrospective multicenter study performed in 10 pediatric rheumatology services of São Paulo state, Brazil. Anti-P antibodies were measured by ELISA in 228 cSLE patients. Results Anti-P antibodies were observed in 61/228 (27%) cSLE patients. Frequencies of cumulative lymphadenopathy (29% vs. 15%, p = 0.014), acute confusional state (13% vs. 5%, p = 0.041), mood disorder (18% vs. 8%, p = 0.041), autoimmune hemolytic anemia (34% vs. 15%, p = 0.001), as well as presence of anti-Sm (67% vs. 40%, p = 0.001), anti-RNP (39% vs. 21%, p = 0.012) and anti-Ro/SSA antibodies (43% vs. 25%, p = 0.016) were significantly higher in cSLE patients with anti-P antibodies compared to those without these autoantibodies. A multiple regression model revealed that anti-P antibodies were associated with autoimmune hemolytic anemia (odds ratio (OR) = 2.758, 95% confidence interval (CI): 1.304-5.833, p = 0.008) and anti-Sm antibody (OR = 2.719, 95% CI: 1.365-5.418, p = 0.004). The SLICC/ACR damage index was comparable in patients with and without anti-P antibodies ( p = 0.780). Conclusions The novel association of anti-P antibodies and autoimmune hemolytic anemia was evidenced in cSLE patients and further studies are necessary to determine if anti-P titers may vary with this hematological manifestation.

Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups.

Objective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (≥6 and <12 years) and group C adolescent (≥12 and <18 years). Methods An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was significantly higher in group A compared to groups B and C (8.3 (0.1-23.4) vs 6.2 (0-17) vs 3.3 (0-14.6) years, p < 0.0001). The median Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) (0 (0-9) vs 0 (0-6) vs 0 (0-7), p = 0.065) was comparable in the three groups. Further analysis of organ/system damage revealed that frequencies of neuropsychiatric (21% vs 10% vs 7%, p = 0.007), skin (10% vs 1% vs 3%, p = 0.002) and peripheral vascular involvements (5% vs 3% vs 0.3%, p = 0.008) were more often observed in group A compared to groups B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia, and autoimmune hemolytic anemia were similar in all groups ( p > 0.05). Mortality rate was significantly higher in group A compared to groups B and C (15% vs 10% vs 6%, p = 0.028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity. Conclusions This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes. This group was characterized by higher mortality rate and neuropsychiatric/vascular/skin organ damage in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.

Wernicke's encephalopathy mimicking neuropsychiatric symptoms in patients with systemic lupus erythematosus: a report of three cases and literature review.

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease that involves many organs and systems. Nervous system involvement in SLE encompasses neurological and psychiatric disorders, and remains a diagnostic and therapeutic challenge. Wernicke's encephalopathy (WE) is a neurological disorder that occurs as a consequence of thiamine deficiency, and its clinical presentation resembles the neuropsychiatric events attributed to SLE (NPSLE). Differentiation between these two entities is crucial because their treatment differs greatly and can change prognosis. We describe three cases of patients with SLE who presented with initial clinical findings suggestive of NPSLE that, at the end of a thorough clinical investigation, were actually found to represent WE. In all of these cases, treatment with thiamine resulted in significant improvement. WE should be considered as a differential diagnosis in SLE patients with neuropsychiatric signs and symptoms, especially when risk factors for thiamine deficiency are present.

Comparison of estimates of body fat content in childhood-onset systemic lupus erythematosus.

Objective We aimed to compare estimates of body fat content with respect to their ability to predict the percentage of body fat, confirmed by dual-energy X-ray absorptiometry scans in childhood-onset systemic lupus erythematosus. Methods We included 64 consecutive childhood-onset systemic lupus erythematosus patients and 64 healthy age and sex-matched controls in a cross-sectional study. Anthropometric data, body mass index and body adiposity index were calculated for all subjects. Childhood-onset systemic lupus erythematosus patients were further assessed for clinical and laboratory childhood-onset systemic lupus erythematosus manifestations and fat mass, lean mass and percentage of body fat evaluated by dual-energy X-ray absorptiometry. Results Elevated waist/hip ratio was observed in childhood-onset systemic lupus erythematosus patients when compared to controls ( p < 0.001). We did not find differences between body mass index and body adiposity index classification in childhood-onset systemic lupus erythematosus patients and controls. Using dual-energy X-ray absorptiometry as gold standard we observed that all indirect estimates of body fat were correlated with whole body fat mass. We observed a correlation between height and cumulative corticosteroid dose adjusted by weight ( r = 0.429, p = 0.005) in childhood-onset systemic lupus erythematosus. On whole body analysis we observed a correlation between lean mass and ACR Damage Index scores ( r = -0.395; p = 0.019); percentage of body fat and adjusted Systemic Lupus Erythematosus Disease Activity Index ( r = 0.402; p = 0.008), disease duration ( r = -0.370; p = 0.012). On trunk analysis we observed a correlation between lean mass and ACR Damage Index ( r = -0.319; p = 0.042); percentage of body fat with adjusted Systemic Lupus Erythematosus Disease Activity Index ( r = 0.402; p = 0.005), disease duration ( r = -0.408; p = 0.005). Conclusions This is the first study analyzing body adiposity index in childhood-onset systemic lupus erythematosus patients. We observed that all indirect estimates of body fat were correlated with whole body fat mass. This study shows that we should not replace body mass index by body adiposity index to evaluating fat levels in childhood-onset systemic lupus erythematosus. In consideration of the importance of overweight classification in cardiovascular diseases, any direct estimates of body fat can be used in an attempt to improve the prognosis of patients. Note We believe that we have presented evidence of body adiposity index accuracy in childhood-onset systemic lupus erythematosus patients but further research on the generalizability of body adiposity index to other patient groups needs to be done.

Abnormality in hippocampal signal intensity predicts atrophy in patients with systemic lupus erythematosus.

Objectives To quantify signal abnormalities in the hippocampus (Hsig) of patients with systemic lupus erythematosus (SLE) and to determine if Hsig predict hippocampal atrophy (HA) in SLE. Methods We included all SLE patients and healthy age- and sex-matched individuals with two magnetic resonance imaging (MRI) scans performed with a minimum of 1 year interval. All individuals underwent a standardized neuropsychological evaluation. Individual results were converted into standard scores and compared to normative data. SLE patients were additionally assessed for disease activity (SLE Disease Activity Index (SLEDAI)), damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)), and the presence of antiphospholipid antibodies. MRI was performed on an Elscint 2 T scanner and T1 inversion recovery and T2 coronal images were used for analysis. Volumetric (HV) and signal quantification (Hsig) were determined by standardized protocols. Results We included 54 SLE patients (48 women; mean age 32.2 ± 10.56 years). Hsig were found at study entry in 15 (45.5%) patients. Hsig in the body and tail of non-atrophic hippocampi correlated with progression of volume loss during the follow-up period ( r = 0.8, p < 0.001). The presence of Hsig in the head of atrophic hippocampi correlated with progression of HA ( r = 0.73, p = 0.005) during the same period. No correlation of Hsig and disease activity or prednisone dose was observed. Conclusion HA is frequently observed in SLE patients and volume loss is progressive in a subgroup of patients. The evaluation of Hsig is an easy tool to determine patients that may have progressive hippocampal volume loss and should be followed more closely with MRI and cognitive evaluation.

Posterior reversible encephalopathy syndrome and association with systemic lupus erythematosus.

INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a neurological complex disorder with many clinical associations and causative factors. It is important to recognize this condition because early diagnosis and treatment usually result in its complete resolution, radiological imaging becoming the key for the correct diagnosis. METHODS: We retrospectively reviewed charts and magnetic resonance imaging findings in the University of Campinas from January 2005 to July 2015, selecting three cases of patients with systemic lupus erythematosus syndrome who developed PRES, for whom risk factors, characteristics, magnetic resonance imaging findings and neurological resolution were analyzed. We also conducted a review of the English-language literature. RESULTS: The three cases had neurological symptoms like acute onset of headache, altered mental status, cortical blindness and seizures. Brain magnetic resonance imaging demonstrated posterior cortical and white matter alterations involving posterior brain territories, which were more conspicuous on T2-weighted and fluid-attenuated inversion recovery. Spectroscopy, diffusion-weighted imaging and susceptibility-weighted imaging were also important for neuroradiological evaluation. Immunosuppressive drugs were taken in all cases. Partial clinical and radiological recovery was observed in two cases, and complete resolution was observed in the third patient. LITERATURE REVIEW: We found 52 cases of PRES in systemic lupus erythematosus patients. Almost all patients were women 94%, ranging from 8 to 62 years old. Posterior brain territory involvements were found in 98% of patients. Hemorrhagic complications involved 26% of patients, becoming a risk factor for clinical sequels. The total percentage of patients with no complete resolution of radiological findings on follow-up images was 27.5%. DISCUSSION: In patients with autoimmune disorders, endothelial dysfunction may occur secondary to autoimmunity and the use of cytotoxic drugs, supposedly facilitating the occurrence of more severe PRES. The hypothesis that patients with autoimmune diseases have a propensity to develop non-reversible lesions has been raised.

Gender differences in systemic lupus erythematosus concerning anxiety, depression and quality of life.

Several studies have demonstrated a high prevalence of depression and anxiety in patients with systemic lupus erythematosus (SLE); however, few data address gender differences regarding these manifestations. This study aimed to investigate gender differences in the prevalence of depressive and anxiety symptoms, and their effect on the quality of life (QOL) of male and female SLE patients. This study included 54 male SLE patients, 54 female SLE patients, 54 male controls and 54 female controls. Depressive symptoms were assessed using the Beck Depression Inventory (BDI), the Center for Epidemiologic Studies Depression Scale (CES-D) and the Hospital Anxiety and Depression Scale (HADS); the anxiety symptoms were examined using HADS. We used the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36) to assess QOL. Depressive symptoms were found in 22.2% of BDI respondents, 24.1% of CES-D respondents and 13% of HADS-D respondents who were male SLE patients; while in the female SLE patient group, they were found in 38.9% of BDI respondents (p = 0.063), 51.9% of CES-D respondents (p = 0.653) and 31.5% of HADS-D respondents (p = 0.003). Anxiety symptoms were found in 16.7% of the male SLE patients and 38.9% of the female SLE patients (p = 0.024). Lower scores on the SF-36 (for QOL) were found in both male and female SLE patients with depression and anxiety symptoms. In conclusion, we observed significant gender differences regarding the prevalence of depressive and anxiety symptoms in patients with SLE, with significantly higher values in the female group. The presence of these symptoms appears to have a negative effect on the QOL of patients of both genders.

Loss of cerebral white matter in primary Sjögren's syndrome: a controlled volumetric magnetic resonance imaging study.

BACKGROUND AND PURPOSE: Although brain involvement is common in primary Sjögren's syndrome (pSS), results from cerebral imaging studies are inconsistent. This study aimed to perform both voxel-wise and global brain volume analyses in a nearly population-based pSS cohort to explore whether the patients displayed any focal or diffuse volume differences compared with healthy subjects. METHODS: Global grey matter (GM) and white matter (WM) volumes were measured and compared in 60 patients with pSS and 60 age- and gender-matched healthy subjects. Regression models were constructed with potential explanatory variables for GM and WM volumes. In the same groups, voxel-wise morphometric analyses were performed. RESULTS: In analyses of global GM and WM, the patients had lower WM volumes than healthy subjects (540 ± 63 cm(3) vs. 564 ± 56 cm(3), P = 0.02), but no differences in GM. Voxel-wise analyses displayed no localized areas of GM or WM volume differences between pSS patients and healthy subjects. CONCLUSION: Individuals with pSS have a diffuse reduction of cerebral WM but no localized loss of WM or GM. This indicates a general deleterious effect on WM due to pSS itself.

Validation of the Portuguese Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY) in Brazil.

BACKGROUND AND OBJECTIVE: Simple Measure of the Impact of Lupus Erythematosus in Youngsters (SMILEY) is a health-related quality of life (HRQOL) assessment tool for pediatric systemic lupus erythematosus (SLE), which has been translated into Portuguese for Brazil. We are reporting preliminary data on cross-cultural validation and reliability of SMILEY in Portuguese (Brazil). METHODS: In this multi-center cross-sectional study, Brazilian children and adolescents 5-18 years of age with SLE and parents participated. Children and parents completed child and parent reports of Portuguese SMILEY and Portuguese Pediatric Quality of Life Inventory (PedsQL™) Generic and Rheumatology modules. Parents also completed the Childhood Health Assessment Questionnaire (CHAQ). Physicians completed the SLE disease activity index (SLEDAI), Physician's Global Assessment of disease activity (PGA) and Systemic Lupus Erythematosus International Collaborating Clinics ACR Damage Index (SDI). RESULTS: 99 subjects (84 girls) were enrolled; 93 children and 97 parents filled out the SMILEY scale. Subjects found SMILEY relevant and easy to understand and completed SMILEY in 5-15 minutes. Brazilian SMILEY was found to have good psychometric properties (validity and reliability), and the child-parent agreement was moderate. CONCLUSION: SMILEY may eventually be used routinely as a research/clinical tool in Brazil. It may be also adapted for other Portuguese-speaking nations offering critical information regarding the effect of SLE on HRQOL for children with SLE.