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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. [ABSTRACT FROM AUTHOR]

Background: Systemic lupus erythematosus (SLE) often mimics symptoms of other diseases, and the interval between symptom onset and diagnosis may be long in some of these patients. Aims: To describe the characteristics associated with the time to SLE diagnosis and its impact on damage accrual and mortality in patients with SLE from a Latin American inception cohort., Methods: Patients were from a multi-ethnic, multi-national Latin-American SLE inception cohort. All participating centers had specialized lupus clinics. Socio-demographic, clinical/laboratory, disease activity, damage, and mortality between those with a longer and a shorter time to diagnosis were compared using descriptive statistical tests. Multivariable Cox regression models with damage accrual and mortality as the end points were performed, adjusting for age at SLE diagnosis, gender, ethnicity, level of education, and highest dose of prednisone for damage accrual, plus highest dose of prednisone, baseline SLEDAI, and baseline SDI for mortality., Results: Of the 1437 included in these analyses, the median time to diagnosis was 6.0 months (Q1-Q3 2.4-16.2); in 721 (50.2%) the time to diagnosis was longer than 6 months. Patients whose diagnosis took longer than 6 months were more frequently female, older at diagnosis, of Mestizo ethnicity, not having medical insurance, and having "non-classic" SLE symptoms. Longer time to diagnosis had no impact on either damage accrual (HR 1.09, 95% CI 0.93-1.28, p = 0.300) or mortality (HR 1.37, 95% CI 0.88-2.12, p = 0.200)., Conclusions: In this inception cohort, a maximum time of 24 months with a median of 6 months to SLE diagnosis had no apparent negative impact on disease outcomes (damage accrual and mortality)., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Introduction: Factors related to presentation of neuropsychiatric (NP) SLE manifestations, early in the course of the disease, and during follow up have not been clearly established., Purpose: To identify disease and non-disease related factors associated with NP manifestations in early SLE., Methods: We included 1193 patients from the GLADEL inception cohort free of NP involvement at cohort entry. We evaluated the association of demographic, clinical and laboratory data with NP involvement during follow-up., Statistical Methods: Independent factors associated with NP involvement were identified using a multivariable Cox regression model., Results: Factors independently associated with NP manifestations were: mestizo ethnicity (HR 1.701, 95% CI 1.282-2.258, p = 0.0002), myalgias/myositis (HR 1.832, 95% CI 1.335-2.515, p = 0.0002), pneumonitis (HR 2.476, 95% CI 1.085-5.648, p = 0.0312), shrinking lung (HR 2.428, 95% CI 1.074-5.493, p = 0.0331) and hemolytic anemia (HR 1.629, 95% CI 1.130-2.347, p = 0.0089). Longer disease duration at cohort entry (13 to 24 months) was associated with a lower risk of developing NP manifestations (HR 0.642, 95% CI 0.441-0.934, p = 0.0206)., Conclusions: Patients with myalgias/myositis, pneumonitis, shrinking lung and hemolytic anemia are at higher risk of NP involvement, whereas longer disease duration at cohort entry is associated with a lower risk of developing NP involvement.

Aim: The aim of this study was to identify factors predictive of serious infections over time in patients with systemic lupus erythematosus (SLE)., Methods: A multi-ethnic, multi-national Latin American SLE cohort was studied. Serious infection was defined as one that required hospitalization, occurred during a hospitalization or led to death. Potential predictors included were sociodemographic factors, clinical manifestations (per organ involved, lymphopenia and leukopenia, independently) and previous infections at baseline. Disease activity (SLEDAI), damage (SLICC/ACR Damage Index), non-serious infections, glucocorticoids, antimalarials (users and non-users), and immunosuppressive drugs use; the last six variables were examined as time-dependent covariates. Cox regression models were used to evaluate the predictors of serious infections using a backward elimination procedure. Univariable and multivariable analyses were performed., Results: Of the 1243 patients included, 1116 (89.8%) were female. The median (interquartile range) age at diagnosis and follow-up time were 27 (20-37) years and 47.8 (17.9-68.6) months, respectively. The incidence rate of serious infections was 3.8 cases per 100 person-years. Antimalarial use (hazard ratio: 0.69; 95% confidence interval (CI): 0.48-0.99; p  = 0.0440) was protective, while doses of prednisone >15 and ≤60 mg/day (hazard ratio: 4.18; 95 %CI: 1.69-10.31; p  = 0.0019) and >60 mg/day (hazard ratio: 4.71; 95% CI: 1.35-16.49; p  = 0.0153), use of methylprednisolone pulses (hazard ratio: 1.53; 95% CI: 1.10-2.13; p  = 0.0124), increase in disease activity (hazard ratio: 1.03; 95% CI: 1.01-1.04; p  = 0.0016) and damage accrual (hazard ratio: 1.22; 95% CI: 1.11-1.34; p  < 0.0001) were predictive factors of serious infections., Conclusions: Over time, prednisone doses higher than 15 mg/day, use of methylprednisolone pulses, increase in disease activity and damage accrual were predictive of infections, whereas antimalarial use was protective against them in SLE patients.

Introduction: Neuropsychiatric systemic lupus erythematosus (NPSLE) is characterized by a heterogeneity of clinical manifestations. The absence of diagnostic criteria and the lack of clinical trials is a challenge in clinical practice. Areas covered: A literature review was performed to describe epidemiology, characterization (clinical, immunological, and imaging), diagnosis and treatment of NPSLE. Classification criteria have been the first step towards a uniform definition. More recently, different attribution models have been developed to help to determine if the NP event is due to SLE. Disease activity is a major risk factor for NP events. Cytokines and autoantibodies are associated with NP events, however, only a few studies have identified risk factors for individual NP events. Expert opinion: Further research needs to search for and validate biomarkers for NPSLE and individual NP events, including neuroimaging findings, attribution models, and serologic markers. This will be a fundamental step in planning randomized control trials in the treatment of NPSLE to improve outcome.

Systemic lupus erythematosus (SLE), presenting with new onset or worsening neuropsychiatric (NP) symptoms, is a challenge in clinical practice. Mimickers such as infections, drug-induced side effects, metabolic abnormalities, malignancies, and alcohol-related disorders have to be excluded, before attributing the manifestations to disease activity. Proper diagnosis is essential to guide adequate management and reduce morbidity and mortality. In this review article, we will highlight clinical, laboratorial, and neuroradiological features that are helpful to assist in the differential diagnosis., (Copyright © 2019. Published by Elsevier Ltd.)

Neuropsychiatric lupus (NPSLE) is described in 12-95% of patients with systemic lupus erythematosus (SLE). Anxiety disorders are among the most frequent manifestations of NPSLE, occurring in 4-85% of these patients. Several diagnostic tools, such as Hospital Anxiety and Depression Scale (HADS), have been used to assess anxiety in clinical studies in SLE, but there is a lack of data on the performance of these questionnaires in the disease. This study aimed to assess the performance of HADS for the detection of anxiety in male and female patients with SLE, also investigating possible gender differences in this aspect. This study included 54 male SLE patients and 54 female SLE patients. The Diagnostic Criteria for Generalized Anxiety Disorder of the 5th edition of the Diagnostic and Statistical Manual of Mental Disorders was used as gold-standard method to assess the performance of HADS for detecting anxiety in SLE patients. HADS presented sensitivity of 88.9% and specificity of 92.6%, with positive and negative predictive values of 80.0 and 96.1%, respectively. The HADS accuracy in total sample was 92.6%, with Kappa coefficient equal to 0.5794 (95% CI 0.3894-0.7695). No significant differences were observed between female and male groups regarding the performance of HADS for diagnosing anxiety.

Objectives The objectives of this study were to examine the demographic and clinical features associated with the occurrence of pleuropulmonary manifestations, the predictive factors of their occurrence and their impact on mortality in systemic lupus erythematosus (SLE) patients. Materials and methods The association of pleuropulmonary manifestations with demographic and clinical features, the predictive factors of their occurrence and their impact on mortality were examined in GLADEL patients by appropriate univariable and multivariable analyses. Results At least one pleuropulmonary manifestation occurred in 421 of the 1480 SLE patients (28.4%), pleurisy being the most frequent (24.0%). Age at SLE onset ≥30 years (OR 1.42; 95% CI 1.10-1.83), the presence of lower respiratory tract infection (OR 3.19; 95% CI 2.05-4.96), non-ischemic heart disease (OR 3.17; 95% CI 2.41-4.18), ischemic heart disease (OR 3.39; 95% CI 2.08-5.54), systemic (OR 2.00; 95% CI 1.37-2.91), ocular (OR 1.58; 95% CI 1.16-2.14) and renal manifestations (OR 1.44; 95% CI 1.09-1.83) were associated with pleuropulmonary manifestations, whereas cutaneous manifestations were negatively associated (OR 0.47; 95% CI 0.29-0.76). Non-ischemic heart disease (HR 2.24; 95% CI 1.63-3.09), SDI scores ≥1 (OR 1.54; 95% CI 1.10-2.17) and anti-La antibody positivity (OR 2.51; 95% CI 1.39-4.57) independently predicted their subsequent occurrence. Cutaneous manifestations were protective of the subsequent occurrence of pleuropulmonary manifestations (HR 0.62; 95% CI 0.43-0.90). Pleuropulmonary manifestations independently contributed a decreased survival (HR: 2.79 95% CI 1.80-4.31). Conclusion Pleuropulmonary manifestations are frequent in SLE, particularly pleuritis. Older age, respiratory tract infection, cardiac, systemic and renal involvement were associated with them, whereas cutaneous manifestations were negatively associated. Cardiac compromise, SDI scores ≥1 and anti-La positivity at disease onset were predictive of their subsequent occurrence, whereas cutaneous manifestations were protective. They independently contributed to a decreased survival in these patients.

Aims: To determine the factors predictive of disease activity early in the course of SLE (baseline visit)., Methods: Patients from GLADEL, a multi-national, multi-ethnic, Latin-American lupus cohort were included. Disease activity was evaluated at baseline with the SLEDAI score. Demographic characteristics (age at diagnosis, gender, ethnicity, marital status, educational level, medical coverage and socioeconomic status) were assessed. Disease duration was defined as the time between the fourth ACR criterion and baseline. Time to criteria accrual was defined as the interval between the first and fourth ACR criterion. Use of glucocorticoids was recorded as the highest dose received before the baseline visit. Antimalarials and immunosuppressive drugs were recorded as use or not use. Univariable and multivariable analysis were performed. Model selection was based on backward elimination., Results: One thousand two hundred sixty-eight patients were included; 1136 (89.6%) of them were female. Mean age at diagnosis was 29.2 (SD: 12.3) years. Five hundred sixty-five (44.6%) were Mestizo, 539 (42.5%) were Caucasians and 164 (12.9%) were African-Latin-Americans. The mean SLEDAI at baseline was 10.9 (SD: 8.4). Longer time between first and fourth ACR criterion, medical coverage, a dose of prednisone between 15 and 60mg/d, and the use of antimalarials were factors protective of disease activity, while Mestizo and African-Latin-American ethnicities were predictive factors., Conclusions: Mestizo and African-Latin-American ethnicities were predictive whereas antimalarial use, medical coverage, and longer time to criteria accrual were protective of higher disease activity early in the disease course., (Copyright © 2017 Elsevier Inc. All rights reserved.)

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.

Objectives: The objective of this study was to examine whether early discoid lupus erythematosus (DLE) would be a protective factor for further lupus nephritis in patients with systemic lupus erythematosus (SLE)., Methods: We studied SLE patients from GLADEL, an inception longitudinal cohort from nine Latin American countries. The main predictor was DLE onset, which was defined as physician-documented DLE at SLE diagnosis. The outcome was time from the diagnosis of SLE to new lupus nephritis. Univariate and multivariate survival analyses were conducted to examine the association of DLE onset with time to lupus nephritis., Results: Among 845 GLADEL patients, 204 (24.1%) developed lupus nephritis after SLE diagnosis. Of them, 10 (4.9%) had DLE onset, compared to 83 (12.9%) in the group of 641 patients that remained free of lupus nephritis (hazard ratio 0.39; P = 0.0033). The cumulative proportion of lupus nephritis at 1 and 5 years since SLE diagnosis was 6% and 14%, respectively, in the DLE onset group, compared to 14% and 29% in those without DLE (P = 0.0023). DLE onset was independently associated with a lower risk of lupus nephritis, after controlling for sociodemographic factors and disease severity at diagnosis (hazard ratio 0.38; 95% confidence interval 0.20-0.71)., Conclusions: Our data indicate that DLE onset reduces the risk of further lupus nephritis in patients with SLE, independently of other factors such as age, ethnicity, disease activity, and organ damage. These findings have relevant prognosis implications for SLE patients and their clinicians. Further studies are warranted to unravel the biological and environmental pathways associated with the protective role of DLE against renal disease in patients with SLE., (© The Author(s) 2016.)

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., (© The Author(s) 2016.)

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.

Aim: To describe the main causes and factors associated with mortality in community-dwelling older adults in a county where the public health system covers most of the population., Methods: We analyzed data from an existing cross-sectional study of 2209 participants (age ≥60 years) in a city in southeast Brazil where 92% of the population is served by a public system of primary care. Over a period of 7 years, 386 participants died and were included in the sample. We assessed the impacts that dependence on others for basic activities of daily living and instrumental activities of daily living, Geriatric Depression Scale scores, and health history have on mortality., Results: The participants' mean age was 75.2 years (SD 8.2); 51.7% of the participants were women, and 51.3% had depressive symptoms. The main causes of death were circulatory diseases (40.3%), cancer (19.8%) and respiratory diseases (13.5%). Multivariate analysis showed that, taken together, the use of more than four medications per day, smoking, lower income, older age and dependence on others for a greater number of instrumental activities of daily living predicted death in this population., Conclusions: Understanding the factors that are associated with mortality can facilitate understanding, and aid in developing policies regarding primary care for the elderly. Geriatr Gerontol Int 2016; 16: 804-809., (© 2015 Japan Geriatrics Society.)

Objective: To describe clinical, laboratory, radiological and progression characteristics of myelopathy in systemic lupus erythematosus (SLE)., Patients and Methods: A retrospective analysis was performed on a cohort of 1193 patients with SLE (ACR criteria) in order to identify patients with myelopathy (neuropsychiatric ACR). Disease activity was assessed by the SLE activity index (SLEDAI) on the date of the event and functional capacity was assessed by the Expanded Disability Status Scale (EDSS) at the last visit., Results: We identified 14 (1.2%) patients with myelopathy. All were women with a mean age of 30±11.5 years. Myelopathy occurred at the diagnosis of SLE in four (28%) patients; and nine (64%) patients had another type of neuropsychiatric manifestation associated. Neurological recurrence was observed in one (7%) patient. Disease activity was observed in 2 (14%) patients. Cerebrospinal fluid presented pleocytosis on 7 (53%) patients; antiphospholipid antibodies were positive in 5 (45%). Magnetic resonance imaging (MRI) showed T2 hyperintensity with a predominance of longitudinal involvement in 6 (86%) patients. Most were treated with intravenous corticosteroids and cyclophosphamide. No patient had full recovery and four (36%) had high EDSS scores. Three (21%) patients died from sepsis early in the course of their myelopathy, during or after immunosuppressive therapy., Conclusions: Myelopathy occurred in 14 (1.2%) of the patients in our cohort and this may be the first manifestation of the disease occurring independently of systemic disease activity. Although rare, myelopathy shows great morbidity and mortality, can be recurrent and MRI is critical for diagnosis., (Copyright © 2016. Published by Elsevier Editora Ltda.)

Background: Tumor necrosis factor alpha (TNF-α) is deeply related to pathogenesis of neurodevelopmental disorders, especially depression. The aim of this study was to explore potential relationships between sera TNF-α levels and mood and anxiety disorders in systemic lupus erythematosus (SLE) patients., Methods: We included 153 consecutive SLE patients (women 148; median age 30; range 10-62) and 40 (women 37; mean age 28.5; range 12-59) age- and sex-matched healthy controls. Mood and anxiety disorders were determined through Beck Depression and Beck Anxiety Inventory. SLE patients were further assessed for clinical and laboratory SLE manifestations. TNF-α levels were measured by enzyme-linked immunosorbent assay using commercial kits., Results: Depressive symptoms were identified in 70 (45.7 %) SLE patients and in 10 (25 %) healthy controls (p < 0.001). Anxiety symptoms were identified in 93 (60.7 %) SLE patients and in 16 controls (40 %) (p < 0.001). Sera TNF-α levels were increased in SLE patients with depressive symptoms (p < 0.001) and with anxiety symptoms (p = 0.014). A direct correlation between the severity of depressive symptoms and sera TNF-α levels (r = 0.22; p = 0.003) was observed. TNF-α levels were significantly increased in patients with active disease (p = 0.012). In addition, we observed a correlation between sera TNF-α levels and disease activity (r = 0.28; p = 0.008). In the multivariate analysis, sera TNF-α levels were independently associated with depressive symptoms (t = 3.28; 95 % CI 1.08-2.2; p = 0.002)., Conclusions: Sera TNF-α levels are increased in SLE patients with mood and anxiety disorders. In SLE, sera TNF-α levels are independently associated with mood disorders. The etiology of mood disorders is still debated in SLE, but our findings suggest the presence of immunological basis for depression in SLE.

Systemic lupus erythematosus is an autoimmune disease that causes many psychological repercussions that have been studied through qualitative research. These are considered relevant, since they reveal the amplitude experienced by patients. Given this importance, this study aims to map the qualitative production in this theme, derived from studies of experiences of adult patients of both genders and that had used as a tool a semi-structured interview and/or field observations, and had made use of a sampling by a saturation criterion to determine the number of participants in each study. The survey was conducted in Pubmed, Lilacs, Psycinfo e Cochrane databases, searching productions in English and Portuguese idioms published between January 2005 and June 2012. The 19 revised papers that have dealt with patients in the acute phase of the disease showed themes that were categorized into eight topics that contemplated the experienced process at various stages, from the onset of the disease, extending through the knowledge of the diagnosis and the understanding of the manifestations of the disease, drug treatment and general care, evolution and prognosis. The collected papers also point to the difficulty of understanding, of the patients, on what consists the remission phase, revealing also that this is a clinical stage underexplored by psychological studies., (Copyright © 2014 Elsevier Editora Ltda. All rights reserved.)

Purpose: To determine the association between the number of flares systemic lupus erythematosus (SLE) patients experience and damage accrual, independently of other known risk factors., Methods: SLE patients (34 centres, nine Latin American countries) with a recent diagnosis (≤2 years) and ≥3 evaluations were studied. Disease activity was ascertained with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and damage with the SLICC/ACR Damage Index (SDI). Flare was defined as an increase ≥4 points in the SLEDAI between two study visits. An ambidirectional case- crossover design was used to determine the association between the number of flares and damage accrual., Results: 901 patients were eligible for the study; 500 of them (55.5%) experienced at least one flare, being the mean number of flares 0.9 (SD: 1.0). 574 intervals from 251 patients were included in the case-crossover design since they have case and control intervals, whereas, the remaining patients did not. Their mean age at diagnosis was 27.9 years (SD: 11.1), 213 (84.9%) were women. The mean baseline SDI and SLEDAI were 1.3 (1.3) and 13.6 (8.1), respectively. Other features were comparable to those of the entire sample. After adjusting for possible confounding variables, the number of flares, regardless of their severity, was associated with damage accrual (SDI) OR 2.05, 95% CI 1.43 to 2.94, p<0.001 (OR 2.62, 95% CI 1.31 to 5.24, p=0.006 for severe and OR 1.91, 95% CI 1.28 to 2.83, p=0.001 for mild-moderate)., Conclusions: The number of flares patients experience, regardless of their severity, increases the risk of damage accrual, independently of other known risk factors., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Objectives: Rheumatoid arthritis is a polygenically controlled systemic autoimmune disease. Rheumatoid vasculitis is an important extra-articular phenotype of rheumatoid arthritis that can result in deep cutaneous ulcers. The objective of this study was to establish a correlation between the frequency of major histocompatibility complex class I/II alleles and killer immunoglobulin-like receptor genotypes in patients with cutaneous rheumatoid vasculitis., Methods: Using the Scott & Bacon 1984 criteria to diagnose rheumatoid vasculitis and after excluding any other causes such as diabetes, atherosclerosis, adverse drug reactions, infection, and smoking, patients who met the criteria were selected. All of the selected rheumatoid vasculitis patients presented deep cutaneous ulcers. Identification of the major histocompatibility complex class I/II and killer immunoglobulin-like receptor genotypes was performed by polymerase chain reaction assays of samples collected from the 23 rheumatoid vasculitis patients as well as from 80 controls (40 non-rheumatoid vasculitis RA control patients and 40 healthy volunteers)., Results: An association between the presence of the HLA-DRB1*1402 and HLA-DRB1*0101 alleles and cutaneous lesions in rheumatoid vasculitis patients and a correlation between the inhibitor KIR2DL3 and the HLA-C*0802 ligand in rheumatoid vasculitis patients were found., Conclusion: An association was found between the presence of the HLA-DRB1*1402 and HLA-DRB1*0101 alleles and the development of cutaneous lesions in rheumatoid vasculitis patients. Additionally, the HLA-C*0802 ligand protects these individuals from developing cutaneous lesions.

Objective: To develop recommendations for the diagnosis, management and treatment of lupus nephritis in Brazil., Method: Extensive literature review with a selection of papers based on the strength of scientific evidence and opinion of the Commission on Systemic Lupus Erythematosus members, Brazilian Society of Rheumatology., Results and Conclusions: 1) Renal biopsy should be performed whenever possible and if this procedure is indicated; and, when the procedure is not possible, the treatment should be guided with the inference of histologic class. 2) Ideally, measures and precautions should be implemented before starting treatment, with emphasis on attention to the risk of infection. 3) Risks and benefits of treatment should be shared with the patient and his/her family. 4) The use of hydroxychloroquine (preferably) or chloroquine diphosphate is recommended for all patients (unless contraindicated) during induction and maintenance phases. 5) The evaluation of the effectiveness of treatment should be made with objective criteria of response (complete remission/partial remission/refractoriness). 6) ACE inhibitors and/or ARBs are recommended as antiproteinuric agents for all patients (unless contraindicated). 7) The identification of clinical and/or laboratory signs suggestive of proliferative or membranous glomerulonephritis should indicate an immediate implementation of specific therapy, including steroids and an immunosuppressive agent, even though histological confirmation is not possible. 8) Immunosuppressives must be used during at least 36 months, but these medications can be kept for longer periods. Its discontinuation should only be done when the patient achieve and maintain a sustained and complete remission. 9) Lupus nephritis should be considered as refractory when a full or partial remission is not achieved after 12 months of an appropriate treatment, when a new renal biopsy should be considered to assist in identifying the cause of refractoriness and in the therapeutic decision., (Copyright © 2014 Elsevier Editora Ltda. All rights reserved.)

Objective: Evaluate SLE stable patients, without overt respiratory compromise, by means of 6MWT., Casuistic and Methods: Forty-five stable SLE patients were enrolled. The ATS/ERS protocol for 6MWT, was used and two parameters with cut-off points were chosen., Results: Forty-two patients were women. The mean age was 39 ± 11.4 years; mean duration of disease, 121 ± 93.1 months; mean value of MRC, 2 ± 0; mean FVC, 85.9 ± 34.2%; mean FEV1, 67.5 ± 21.6%; mean MIP, 82 ± 58.4%; mean MEP, 78 ± 37.3%; mean heart rate at rest, 75 ± 12.8 bpm; mean respiratory rate at rest, 19 ± 5.3 bpm; mean 6MWD, 478 ± 82 m; mean SpO2 at rest was 98 ± 0.8%; mean fall in SpO2, 4 ± 6 points. When the study population was divided according to the 400-m walk distance cut-off value, the heart rate immediately before the test was significant lower in those participants who walked less than 400 m (p = 0.0043), just like the value of Borg scale (p = 0.0036); according to the presence of saturation ≥ 4, heart rate at the end of the test was significantly higher in those participants who were showing desaturation (p = 0.0170); MEP (p = 0.0282) and 6MWD (p = 0.0291) were significantly lower, and MIP showed a tendency towards being smaller (p = 0.0504). FVC < normal inferior limit was significantly associated with the group with desaturation (p = 0.0274)., Conclusion: Compared to 6MWD, desaturation was better suited to find the patients with the most compromised indexes in respiratory function tests.

Aim: In the elderly population, rheumatic conditions are major causes of pain that restrict participation in activities and mobility, and cause difficulties in the execution of self-care tasks. The present study aimed to analyze the prevalence and factors associated with the self-reported rheumatic diseases and chronic joint symptoms of the elderly., Methods: This transversal epidemiological survey involved 2209 older adults (aged ≥ 60 years). The investigation included sociodemographic factors, anthropometrics, activities of daily living, chronic conditions, medication and quality of life. Univariate and multivariate regression analysis were used for statistical procedures, P ≤ 0.05., Results: The prevalence of rheumatism was 22.7%. Multivariate analysis showed that rheumatism was correlated with the following: female sex (OR = 1.91), high income (OR = 2.34), cardiovascular disease (OR = 1.42), cataracts (OR = 1.39), glucocorticoids (OR = 5.24), other anti-inflammatory medications (OR = 2.24) and pain (OR = 0.983). After adjusting for age and glucocorticoids, an association between cataracts and rheumatism was detected (OR = 1.32). The prevalence of symptoms was 45.6%. Multivariate regression results for symptoms included the following: female sex (OR = 1.40), body mass index ≥ 30.0 kg/m(2) (OR = 3.31), functional capacity (OR = 0.990), general health (OR = 0.993) and pain (OR = 0.981). After adjustment for age and glucocorticoids, an association between cataracts and symptoms was detected (OR = 1.26)., Conclusion: There was a significant association of rheumatism and symptoms with women and high incomes. Obesity was associated with joint symptoms, which in turn were associated with an impaired quality of life. Cataracts and cardiovascular disease were associated with rheumatism. The identification of these characteristics in the elderly will contribute to a better understanding of this systemic disease and should be used to plan effective preventive measures., (© 2013 Japan Geriatrics Society.)

Objective: To determine the serum levels of Th1 (IL-12, IFN-γ,TNF-α) and Th2 (IL-5, IL-6 and IL-10) cytokines in childhood-onset SLE, first-degree relatives and healthy controls. To elucidate their association with disease activity, laboratory and treatment features., Methods: We included 60 consecutive childhood-onset SLE patients [median age 18 years (range 10-37)], 64 first-degree relatives [median 40 (range 28-52)] and 57 healthy [median age 19 years (range 6-30 years)] controls. Controls were age and sex-matched to SLE patients. SLE patients were assessed for clinical and laboratory SLE manifestations, disease activity (SLEDAI), damage (SDI) and current drug exposures. Mood and anxiety disorders were determined through Becks Depression (BDI) and Anxiety Inventory (BAI). Th1 (IL-12, IFN-γ,TNF-α) and Th2 (IL-5, IL-6 and IL-10) cytokines levels were measured by ELISA and compared by non-parametric tests., Results: Serum TNF-α (p=0.004), IL-6 (p=0.007) and IL-10 (p=0.03) levels were increased in childhood-onset SLE patients when compared to first-degree relatives and healthy controls. TNF-α levels were significantly increased in patients with active disease (p=0.014) and correlated directly with SLEDAI scores (r=0.39; p=0.002). IL-12 (p=0.042) and TNF-α (p=0.009) levels were significantly increased in patients with nephritis and TNF-α in patients with depression (p=0.001). No association between cytokine levels and SDI scores or medication was observed., Conclusion: Th1 cytokines may play a role in the pathogenesis of neuropsychiatric and renal manifestations in childhood-onset SLE. The correlation with SLEDAI suggests that TNF-α may be a useful biomarker for disease activity in childhood-onset SLE, however longitudinal studies are necessary to determine if increase of this cytokine may predict flares in childhood-onset SLE., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Objective: To investigate the prevalence of the anti-ribosomal P (anti-P) antibodies in childhood-onset systemic lupus erythematosus patients (cSLE), healthy controls and first degree relatives. To elucidate the association between anti-P and disease activity, laboratory and treatment features in cSLE patients., Methods: We included consecutive SLE patients with disease onset before 16 years. Controls were age- and sex-matched. SLE patients were 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. Mood disorders were determined through Becks Depression and Becks Anxiety Inventory. Anti-P measured by enzyme-linked immunosorbent assay., Results: We included 50 consecutive cSLE patients (mean age of 16.82 ± 3.46 years), 35 first degree relatives (mean age of 38.73 ± 3.89 years) and 20 health control (mean age of 18.3 ± 4.97 years). Anti-P was observed in 13 (26%) cSLE patients and in no first-degree relative (p < 0.01) or control (p < 0.01). Anti-P was more frequently observed in patients with anxiety (p < 0.002). No other clinical, laboratory or treatment features, including SLEDAI and SDI scores were associated with the presence of anti-P in cSLE patients., Conclusion: Anti-P is frequently observed in cSLE patients and was associated with the presence of anxiety in this cohort of cSLE.

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.

Objective: To review the clinical, laboratory and outcome features of Evans syndrome (ES) in systemic lupus erythematosus (SLE) patients., Methods: We reviewed the charts of 953 SLE patients followed up regularly at our service. ES was defined as the presence of hemolytic anemia and thrombocytopenia concomitantly or sequentially. Clinical and laboratory manifestations occurring during the disease course, as well as concomitant diseases and survival was carefully reviewed., Results: We identified ES in 26 of 953 (2.7%) SLE patients. Twenty-three were women with mean age at SLE diagnosis of 25.7 years. Four (15%) patients had disease onset before the age of 16. In the majority of patients (92%), immune thrombocytopenia and AIHA appeared simultaneously at the beginning of SLE. Active features of SLE were a frequent finding concomitant to ES, especially arthritis (77%), malar rash (61.5%), photosensitivity (57.6%), oral ulcers (34.6%), nephritis (73%), serositis (54%), neuropsychiatric (19%) and pulmonary (15%) manifestations. In addition to this multisystemic disease, 34.6% of our patients had an association with another autoimmune disease such as antiphospholipid syndrome. Recurrence of ES was observed in only four (15%) patients. After follow-up time of 8.72 years, 19 patients (73%) were in remission and seven (27%) patients died., Discussion: ES is a rare manifestation in SLE, occurring in patients with severe multisystemic SLE manifestations. Treatment strategies frequently used in SLE contribute to longer disease remission and less frequent exacerbation than observed in the general population with ES., (Copyright © 2011 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.)

The aim of this study was to determine the demographic and clinical characteristics in patients diagnosed with Behçet's disease (BD) in Brazil. We performed a retrospective review of all the patients' records with BD diagnosed from 1988 to 2010 in the Rheumatology Department at the State University of Campinas (UNICAMP). All patients had to fulfill the International Study Group for Behçet's disease diagnostic criteria. Eighty-seven patients were included in the study. The female/male ratio was 1.18:1, and the mean age at the onset of the disease onset was 28.03 ± 7.57 years. Oral aphthosis was the most frequent manifestation (100%). Genital aphthosis was also frequent (77%), followed by pseudofolliculitis (47.67%). Ocular symptoms were present in 80% and neurological manifestations in 31.03% of the patients. Arthralgia was reported in 31.03% and arthritis in 13.79% of the cases. Vascular involvement was seen in 13.95% of the patients. Only 1.14% had gastrointestinal involvement. This series, from a South American country, showed a similar general pattern of the BD to those found in different endemic areas in the world, with a high frequency of ocular and neurological manifestations.

Low-molecular-weight heparin-induced skin necrosis can occur as a clinical feature of heparin-induced thrombocytopenia syndrome. Heparin-induced thrombocytopenia and antiphospholipid syndromes have some clinical features in common, including thrombocytopenia and thrombotic events. We describe a 46-year-old woman who developed extensive necrosis in the breast and other sites secondary to the use of enoxaparin after an elective hysterectomy. During the postoperative period, diagnoses of systemic lupus erythematosus and antiphospholipid syndrome were made because of some clinical and laboratory features (seizure, nephritis, bicytopenia, positive nuclear antibody, and positive antiphospholipid antibodies with a previous thrombotic event). The patient's clinical course improved only after corticosteroid therapy and the suspension of enoxaparin. Heparin-induced thrombocytopenia and antiphospholipid syndromes can have platelet factor 4 as a common denominator in their pathogenesis because platelet factor 4 tetramers can bind β2-glycoprotein molecules. This case suggests that use of low-molecular-weight heparins could be more risky in patients with an underlying immune disease and/or could trigger immune reactions that must be analyzed in larger studies.

Objective: To evaluate the frequency of HLA classes I and II and their association with the cutaneous manifestation of rheumatoid vasculitis (RV) in Brazilian patients., Patients and Methods: During one year we selected 130 patients with rheumatoid arthritis (RA) classified according to the American College of Rheumatology, 1987. All patients underwent a clinical and laboratory questionnaire to exclude other causes of cutaneous vasculopathy (neoplasia, infections, illicit drug use, diabetes mellitus, and tobaccoism). Seventy-three patients with any risk factor for other causes of vasculopathy were excluded. Fifty-seven without risk factors for other causes of vasculopathy were included in the study, 17 with RV according to Scott and Bacon's criteria, 1984. Demographic data, time of RA diagnosis, disease activity (DAS28), presence of rheumatoid factor, and anti-cyclic citrullinated peptide antibodies were analyzed. The HLA alleles were typed using the DNA-amplified polymerase chain reaction with low-resolution hybridization and sequence-specific primers., Results: The comparison between the 40 patients without RV and the 17 patients with RV showed an increased frequency of HLA-B*14 (Pc = 0.168) and HLA-Cw*08 (Pc = 0.084) in patients with RV and an increased frequency of HLA-DRB5*01 (Pc = 0.048) in patients without RV., Conclusion: The HLA-DRB5*01 may confer protection against that extra-articular manifestation of RA.

Musculoskeletal disorders are the major causes of the pain in the elderly population. Rheumatic conditions restrict participation in activities and mobility, as well as cause difficulties in the execution of self-care tasks. The assessment of health related quality of life (HRQOL) is an important indicator of the impact of rheumatic diseases on the physical, mental and social aspects. This study aims to analyze the influence of rheumatic diseases and chronic joint symptoms on the quality of life of the elderly (n=2209) aged 60 years or over. The effects of rheumatism and joint symptoms on quality of life were investigated by the Outcome Study Short-Form Health Survey (SF-36), and this analysis was adjusted for age and sex. The univariate analysis of variance and analysis of covariance (ANOVA and ANCOVAS) were used for statistical procedures, p≤0.05. Rheumatic diseases affected: functional capacity (F(1, 2012))=10.9 and pain (F(1, 2012))=34.77. Joint symptoms affected all components of the SF-36: physical functioning (F(1, 2012))=10.9; physical problems (F(1, 2012))=72.61; pain (F(1, 2012))=164.29; general health (F(1, 2012))=71.95; vitality (F(1, 2012))=55.78; social aspect (F(1, 2012))=73.14; emotional aspect (F(1, 2012))=49.09 and mental health (F(1, 2012))=44.72. There was a significant impact of rheumatic diseases on physical health, and that joint symptoms affected self-evaluations of physical and mental health. These results will contribute to a better understanding of this systemic disease and will be used for planning effective interventions., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Systemic lupus erythematosus (SLE) is a prototypic inflammatory autoimmune disorder characterized by multisystem involvement and fluctuating disease activity. Symptoms range from rather mild manifestations such as rash or arthritis to life-threatening end-organ manifestations. Despite new and improved therapy having positively impacted the prognosis of SLE, a subgroup of patients do not respond to conventional therapy. Moreover, the risk of fatal outcomes and the damaging side effects of immunosuppressive therapies in SLE call for an improvement in the current therapeutic management. New therapeutic approaches are focused on B-cell targets, T-cell downregulation and costimulatory blockade, cytokine inhibition, and the modulation of complement. Several biological agents have been developed, but this encouraging news is associated with several disappointments in trials and provide a timely moment to reflect on biologic therapy in SLE.

Objective: To determine the serum levels of interferon alpha in childhood-onset systemic lupus erythematosus patients, their first-degree relatives and healthy controls and to evaluate the associations between serum interferon alpha and disease activity, laboratory findings and treatment features., Methods: We screened consecutive childhood-onset systemic lupus erythematosus patients in a longitudinal cohort at the pediatric rheumatology unit of the State University of Campinas between 2009 and 2010. All patients demonstrated disease onset before the age of 16. Disease status was assessed according to the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). Interferon alpha levels were measured using an enzyme-linked immunoabsorbent assay., Results: We included 57 childhood-onset systemic lupus erythematosus patients (mean age 17.33 ± 4.50), 64 first-degree relatives (mean age 39.95 ± 5.66), and 57 healthy (mean age 19.30 ± 4.97) controls. Serum interferon alpha levels were significantly increased in childhood-onset systemic lupus erythematosus patients compared to their first-degree relatives and healthy controls. Interferon alpha levels were significantly increased in patients with positive dsDNA antibodies, patients with cutaneous vasculitis, patients with new malar rash and patients who were not receiving medication. Interferon alpha levels correlated with C3 levels and systemic lupus erythematosus Disease Activity Index scores. In addition, we observed an inverse correlation between patient age and interferon alpha levels., Conclusion: Interferon alpha may play a role in the pathogenesis of childhood-onset systemic lupus erythematosus, especially in cutaneous manifestations and dsDNA antibody formation. The observation that interferon alpha levels are increased in patients who are not taking medication should be investigated in longitudinal studies to determine whether elevated interferon alpha levels may predict systemic lupus erythematosus flares.

Introduction: Epstein-Barr virus exposure appears to be an environmental trigger for rheumatoid arthritis that interacts with other risk factors. Relationships among anti-cyclic citrullinated peptide antibodies, the shared epitope, and smoking status have been observed in patients with rheumatoid arthritis from different populations., Objective: To perform an association analysis of anti-Epstein-Barr nuclear antigen-1 antibodies, anti-cyclic citrullinated peptide antibodies, the shared epitope, and smoking status in Brazilian patients with rheumatoid arthritis., Methods: In a case-control study, 140 rheumatoid arthritis patients and 143 healthy volunteers who were matched for age, sex, and ethnicity were recruited. Anti-Epstein-Barr nuclear antigen-1 antibodies and anti-cyclic citrullinated peptide antibodies were examined using an enzyme-linked immunosorbent assay, and shared epitope alleles were identified by genotyping. Smoking information was collected from all subjects. A comparative analysis of anti-Epstein-Barr nuclear antigen-1 antibodies, anti-cyclic citrullinated peptide antibodies, the shared epitope, and smoking status was performed in the patient group. Logistic regression analysis models were used to analyze the risk of rheumatoid arthritis., Results: Anti-Epstein-Barr nuclear antigen-1 antibodies were not associated with anti-cyclic citrullinated peptide antibodies, shared epitope alleles, or smoking status. Anti-cyclic citrullinated peptide antibody positivity was significantly higher in smoking patients with shared epitope alleles (OR = 3.82). In a multivariate logistic regression analysis using stepwise selection, only anti-cyclic citrullinated peptide antibodies were found to be independently associated with rheumatoid arthritis (OR = 247.9)., Conclusion: Anti-Epstein-Barr nuclear antigen-1 antibodies did not increase the risk of rheumatoid arthritis and were not associated with the rheumatoid arthritis risk factors studied. Smoking and shared epitope alleles were correlated with anti-cyclic citrullinated peptide-antibody-positive rheumatoid arthritis. Of the risk factors, only anticyclic citrullinated peptides antibodies were independently associated with rheumatoid arthritis susceptibility.

This study aims to determine the factors that may be related to falls in the elderly assisted by the Family Health Program (FHP). Elderly individuals aged 60 years or over, who were assisted by the FHP primary care system (n=2209), responded to a sociodemographic and health questionnaire. Mental health was evaluated using the Geriatric Depression Scale (GDS) and quality of life was assessed by the Medical Outcome Study Short-Form Health Survey (SF-36). In order to verify which independent variables affected the occurrence of falls, logistic regression analysis was performed. 27.1% of the sample reported one fall during the previous year, and 8.7% were recurrent fallers over the same period. The factors considered in the final model for falls were: age over 80 years-old, female gender, the presence of more than eight associated diseases, need for hospitalization during the previous year and appointments outside the FHP routine, hearing complaints, GDS score over 11 points, and emotional problems SF-36 score between 25 and 74 points. The factors included in the final model for falls can be easily identified and properly overcome by FHP strategy. Patient falling history investigation should thus form part of the FHP team routine. This concern should be reinforced when it comes to women aged 80 years and over., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

A 43-year-old woman reported pain in the right hypochondrium, which had started 3 years before and had been worsening for the past few days. Claudication in the superior and inferior limbs, diffuse myalgia, dyspnea, precordialgia followed by dizziness and visual turbidity were added to the clinical picture. In the physical examination bilateral carotid bruit was observed, abdominal aorta murmur and the decrease of the right radial and left pedis pulses and arterial hypertension with difference in the diastolic pressure between limbs >10 mmHg was also observed. On cardiac catheterisation with aortography, right coronary with proximal parietal irregularities, slight pressure increase in right chambers and pulmonary artery, preserved left ventricle contractility, competent valves, carotid and subclavian partial obstruction, severe narrowing of the abdominal aorta below the diaphragm (80%) and right renal artery significant stenosis were observed. Takayasu's arteritis (TA) diagnosis was established according to the ACR criteria based on the clinical symptomatology, on physical and image test findings. Two years later she presented malar rash, photosensitivity, nephropathy, leukopenia, lymphopenia and hemolytic anemia confirming the systemic lupus erythematosus (SLE) diagnosis. TA coexisting with SLE has rarely been reported.

Elderly caregivers suffer physical and psychological consequences of the act of caring. The objective of this study was to characterize primary caregivers of elderly people in the community and identify the higher impacts of this activity on their life. We interviewed 127 caregivers about sociodemographic characteristics, presence of anxiety/depression (self-reporting questionnaire=SRQ), burden of care (caregiver burden scale=CBS); while their dependents were evaluated using sociodemographic questionnaires, health history, activities of daily living (ADL) scale and geriatric depression scale (GDS-15). The caregivers' mean age was 55.1+/-13.3 years; among them most were women and daughters with up to 4 years of education. The mean time as responsible for the elderly was 86.5+/-96.3 months; 56% divided the caring responsibility and 28.2% had another occupation; 32.3% presented psychoemotional illness. There was a positive correlation between the CBS and the caregiver factors: psychoemotional disorders, time as responsible for the elderly and education level; as well as between the CBS and the elderly: number of activities with dependence, presence of depression and incontinences. The correlation between the elderly's personal income and number of visits received was negative. The identification of high impacts on the caregivers' life would facilitate the professional approach., (Copyright 2009 Elsevier Ireland Ltd. All rights reserved.)

Objective: To analyze the longterm followup of a series of Brazilian patients with undifferentiated spondyloarthritis (uSpA)., Methods: Prospective study analyzing a group of 111 patients with the diagnosis of uSpA, fulfilling the European Spondylarthropathy Study Group and the Amor criteria, who were followed for 5 to 10 years in a single university referral center. Patients had their outcome analyzed at 5, 7, and 10 years., Results: There was a predominance of men (81.1%), white ethnicity (78.4%), and positive HLA-B27 (61.3%), with a mean age at onset of 27.2 years. Twenty-seven patients presented development to ankylosing spondylitis (AS; 24.3%) and 3 to psoriatic arthritis (PsA; 2.7%), while 25 patients (22.5%) went into remission during the followup. Univariate logistic regression analysis revealed that ethnicity, HLA-B27, buttock pain, inflammatory low back pain, ankle involvement, grade I sacroiliitis at the beginning of the study, and the use of sulfasalazine were statistically associated with progression to AS. Multivariate logistic regression analysis revealed that HLA-B27 (p = 0.035, OR 6.720, 95% CI 11.45-39.43) and buttock pain (p = 0.009, OR 6.211, 95% CI 1.591-24.25) were statistically associated with progression to AS., Conclusion: In a longterm followup of 111 Brazilian patients with uSpA, HLA-B27 and buttock pain were significant predictors of progression to a definite disease.

Objective: To determine the relationship between cognitive impairment and employment in patients with systemic lupus erythematosus (SLE)., Methods: A total of 167 consecutive patients with SLE were prospectively evaluated, and underwent standardized neuropsychological tests. Multivariate logistic regression was used to determine the likelihood of being unemployed, adjusting for sociodemographic characteristics, employment status at study entry, disease activity, disease duration, damage scores, and depression., Results: At study entry, 118 (70.7%) of 167 patients were employed. In the multivariate analysis, after adjusting for confounders, unemployment was predicted by the number of cognitive domains impaired (odds ratio [OR] 1.76, 95% confidence interval [95% CI] 1.58-6.72), depression (OR 1.3, 95% CI 1.1-3.1), fewer years of education (OR 0.12, 95% CI 0.02-0.34), and the presence of anticardiolipin antibodies (OR 1.43, 95% CI 1.2-6.1). Complex attention (OR 1.7, 95% CI 1.2-3.6), memory (OR 3.1, 95% CI 1.5-6.2), and executive functions (OR 2.4, 95% CI 1.9-6.1) were predictors of unemployment at study entry. After 3 years, 61 (51.7%) of 118 patients were employed (OR 2.25, 95% CI 1.38-3.68; P = 0.001). The likelihood of being unemployed after 3 years was predicted by the number of cognitive domains impaired (OR 3.8, 95% CI 2.5-7.71), depression (OR 2.31, 95% CI 1.01-5.21), fewer years of education (OR 0.09, 95% CI 0.01-0.42), and the presence of anticardiolipin antibodies (OR 2.1, 95% CI 1.2-4.9). Complex attention (OR 2.2, 95% CI 1.1-3.8), memory (OR 3.6, 95% CI 2.1-6.4), executive functions (OR 1.8, 95% CI 1.2-7.3), and reasoning/problem solving (OR 1.9, 95% CI 1.4-7.2) were associated with unemployment after 3 years., Conclusion: The number of cognitive spheres, and especially attention, memory, and executive functions, are important factors associated with unemployment in patients with SLE.

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.

The aim of this study was to evaluate the frequency and clinical associations of HLA-DR alleles in Brazilian Caucasian patients with polyarteritis nodosa (PAN) or microscopic polyangiitis (MPA). We evaluated 29 Caucasian patients with vasculitis classified as PAN or MPA according to the American College of Rheumatology (ACR) 1990 Criteria, Chapel Hill Consensus Conference (CHCC) nomenclature for vasculitis and EULAR recommendations for conducting clinical studies in systemic vasculitis. HLA-DR alleles were typed using polymerase chain reaction-amplified DNA, hybridized with sequence-specific low resolution primers. DNA obtained from 59 Caucasian healthy blood donors were used as control. In order to evaluate if a specific HLA may have influence on the clinical profile of those diseases, we also divided the patients according to Birmingham vasculitis score (BVAS) and Five-Factors Score (FFS) at the time of diagnosis. Increased frequency of HLA-DRB1*16 (p = 0.023) and DRB4*01 (p = 0.048) was found in patients with higher disease activity at the time of diagnosis (BVAS >/=22). Patients with less severe disease (FFS = 0) had a higher frequency of HLA-DRB1*03 (p = 0.011). Patients with gastrointestinal tract involvement had significantly increased frequency of HLA-DRB1*11 or B1*12 (p = 0.046), B1*13 (p = 0.021) and B3 (p = 0.008). In contrast, patients with renal disease, had higher frequency of DRB1*15 or DRB1*16 (p = 0.035) and B5 (p = 0.035). In the subgroup of patients with MPA, increased frequency of HLA-DRB1*15 was found in patients with BVAS >/=22 (p = 0.038) and FFS >/=1 (p = 0.039) suggesting that this allele is associated with more aggressive disease. Antineutrophil cytoplasmic antibodies (ANCA) negative MPA patients had significantly increased frequency of HLA-DRB1*11 or DRB1*12 when compared to ANCA positive patients (p = 0.023). Our results suggest that HLA-DR alleles may influence PAN and MPA clinical expression and outcome and that in MPA they participate in the mechanisms involved in the development to ANCA.

Neuropsychiatric manifestations are commonly observed in systemic lupus erythematosus (SLE) patients; however, cerebellar involvement has rarely been reported. In the presence of acute cerebellar ataxia, etiologies related (focal edema and ischemia) and not related (infections, malignancy and paraneoplastic syndromes) to lupus have to be considered and they imply different treatment strategies. We report the clinical and radiological features of 3 SLE patients who presented with acute cerebellar ataxia. A review of the literature was performed by documenting cases of cerebellar ataxia in SLE and the importance of neuroimaging in the evaluation of these patients.