Longitudinal comparison of IL-6, IL-10, and IL-12 cytokine profiles in adult and childhood-onset systemic lupus erythematosus.

Objective: To compare the levels of Th1 (IL-12) and Th2 (IL-6 and IL10) cytokines over a two-year period among systemic lupus erythematosus patients with childhood-onset (cSLE), adult-onset (sSLE), and healthy controls, and correlate with their clinical, laboratory, and treatment manifestations. Methods: The study included 63 patients with cSLE [57 (90%) women; mean age 19.7 ± 4.3 years (range = 10-29); mean disease duration 7.3 ± 4.2 years (range 2-15)], 67 patients with aSLE [65 (97%) women; mean age of 39.9 ± 11.8 years (range 21-68); disease duration 7.7 ± 3.1 years (range 4-16)], and 40 healthy controls [36 (90%) women; mean age of 29.6 ± 10 years (range 12-49)]. cSLE and aSLE patients were paired by disease duration. Clinical and laboratory manifestations, disease activity (SLEDAI), cumulative damage (SDI), and current drug exposures were evaluated. Symptoms of anxiety and depression were evaluated by the Beck inventory (BAI and BDI, respectively). Th1 (IL-12) and Th2 (IL-6 and IL-10) cytokines were measured by the ELISA test. Data were collected at four different time points (TI, TII, TIII, and TIV) and compared by non-parametric tests. Results: IL-6 levels were significantly higher in aSLE patients compared to healthy controls at times I, II, and III (TI p = 0.013, TII p = 0.015, TIII p = 0.004, and TIV p = 0.634). However, no difference was observed between cSLE patients and healthy controls (TI p = 0.223, TII p = 0.613, TIII p = 0.341, and TIV p = 0.977). In addition, no difference was observed between aSLE and cSLE patients (TI p = 0.377, TII p = 0.123, TIII p = 0.105, and TIV p = 0.591). The levels of IL-12 were significantly higher in cSLE patients compared to healthy controls at all time points (TI p = 0.04, TII p < 0.001, TIII p = 0.015, and TIV p = 0.021). aSLE patients showed significantly elevated levels when compared to healthy controls at time III and IV (TI p = 0.752, TII p = 0.827, TIII p = 0.011*, and TIV p < 0.001*). cSLE patients showed significantly higher levels than aSLE patients at times I and II (TI p = 0.07*, TII p < 0.001*, TIII p = 0.998, and TIV p = 0.140). In aSLE patients, IL-6 was associated with headache (p = 0.006), arthritis (p = 0.044), and nephritis (p = 0.012); IL-10 was associated with nephritis (p = 0.043), hypocomplementemia (p = 0.001), and disease activity (p = 0.001); in these patients, IL-12 was associated with alopecia (p = 0.025) and leukopenia (p = 0.044). In cSLE patients, IL-6 was associated with arthritis (p = 0.022) and malar rash (p = 0.012). Conclusion: aSLE and cSLE patients with long disease duration present similar levels of cytokines, despite differences in clinical activity patterns over time.

Gastrointestinal involvement in systemic lupus erythematosus: A systematic review.

INTRODUCTION: Gastrointestinal involvement is a common complain observed in 40-60% of systemic lupus erythematosus (SLE) patients. We performed a systematic review of clinically severe and potential life-threatening gastrointestinal manifestations and discuss clinical presentation, pathogenesis and treatment. METHODS: We performed a literature search in English literature using PubMed and Embase from 2000 to December 2020. The following MeSH terms: systemic lupus erythematosus, protein-losing enteropathy, ascites, pancreatitis, vasculitis, intestinal vasculitis, enteritis and diarrhea published in the English literature. RESULTS: We identified 141 studies (case reports, case series and cohort studies). The most frequent presenting symptoms are acute abdominal pain, nausea, and vomiting. Many of the manifestations were associated with disease activity. Histological features are rarely available, but both vasculitis and thrombosis have been described. There is no treatment guideline. The majority of patients were treated with corticosteroids and the most common immunososupressant were azathioprine, cyclophosphamide and mycophenolate. CONCLUSION: Vasculitis and thrombosis may be responsible for severe life-threatening manifestations such as pancreatitis, protein loosing gastroenteritis, acalculous cholecistyitis and enteritis.

Correction to: Active human herpesvirus infections in adults with systemic lupus erythematosus and correlation with the SLEDAI score.

An amendment to this paper has been published and can be accessed via the original article.

Active human herpesvirus infections in adults with systemic lupus erythematosus and correlation with the SLEDAI score.

BACKGROUND: Human herpesviruses (HHVs) are responsible for a significant number of clinical manifestations in systemic lupus erythematous (SLE) patients. The aim of this study was to determine the frequency of active HHV infections in SLE patients and correlating them with disease activity. METHODS: Serum samples were collected from 71 SLE patients and their DNAs were extracted and analyzed to detect HHV-DNA viruses using the nucleic acid amplification technique. RESULTS: Fifteen out of the 71 (21.1%) patients tested positive for the HHV-DNA virus. Of them, 11/15 HHV-DNA-positive patients (73.3%) had SLE activity index (SLEDAI - Systemic Lupus Erythematosus Disease Activity Index) ≥8 (p = 0.0001). Active HCMV infection was the mostly frequently observed infection, occurring in 6/15 patients (40%). The frequencies of other active viral infections were 22% for HSV-1, 16.7% for HHV-7, and 5.5% for HSV-2. Viral coinfection (two or more viruses detected in the same sample) occurred in three patients (16.7%). Active HHV infections in SLE patients are more frequent in those with active SLE (≥8), who is at high risk of HHV reactivation and HCMV disease. CONCLUSION: Viral surveillance is important to identify active HHV infections that can cause clinical symptoms and other complication in SLE patients.

Active human herpesvirus infections in adults with systemic lupus erythematosus and correlation with the SLEDAI score

Abstract Background: Human herpesviruses (HHVs) are responsible for a significant number of clinical manifestations in systemic lupus erythematous (SLE) patients. The aim of this study was to determine the frequency of active HHV infections in SLE patients and correlating them with disease activity. Methods: Serum samples were collected from 71 SLE patients and their DNAs were extracted and analyzed to detect HHV-DNA viruses using the nucleic acid amplification technique. Results: Fifteen out of the 71 (21.1%) patients tested positive for the HHV-DNA virus. Of them, 11/15 HHV-DNA-positive patients (73.3%) had SLE activity index (SLEDAI - Systemic Lupus Erythematosus Disease Activity Index) ≥8 (p = 0.0001). Active HCMV infection was the mostly frequently observed infection, occurring in 6/15 patients (40%). The frequencies of other active viral infections were 22% for HSV-1, 16.7% for HHV-7, and 5.5% for HSV-2. Viral coinfection (two or more viruses detected in the same sample) occurred in three patients (16.7%). Active HHV infections in SLE patients are more frequent in those with active SLE (≥8), who is at high risk of HHV reactivation and HCMV disease. Conclusion: Viral surveillance is important to identify active HHV infections that can cause clinical symptoms and other complication in SLE patients.

Systemic Lupus Erythematosus and Pregnancy: A Single-Center Observational Study of 69 Pregnancies.

OBJECTIVE: To evaluate the effects of pregnancy in systemic lupus erythematosus (SLE) patients. METHODS: The present article is a retrospective cohort study. Data were collected from medical records of pregnant women with SLE from January 2002 to December 2012 at Universidade Estadual de Campinas, in the city of Campinas, state of São Paulo, Brazil. Systemic lupus erythematosus and disease activity were defined according to the American College of Rheumatology and the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) criteria respectively. The means, standard deviations (SDs), percentages and correlations were performed using the SAS software, version 9.4 (SAS Institute Inc., Cary, NC, US). RESULTS: We obtained data from 69 pregnancies in 58 women. During pregnancy, a new flare was observed in 39.2% (n = 27). The manifestations were most common in patients with prior kidney disease, and mainly occurred during the third quarter and the puerperium. Renal activity occurred in 24.6% (n = 17), and serious activity, in 16% (n = 11). Of all deliveries, 75% (n = 48) were by cesarean section. Two maternal deaths occurred (3%). Preterm birth was the main complication in the newborns. The abortion rate was 8.7%. Severe SLEDAI during pregnancy was associated with prematurity (100%) and perinatal death (54%). CONCLUSION: The maternal-fetal outcome is worse in SLE when the women experience a flare during pregnancy. The best maternal-fetal outcomes occur when the disease is in remission for at least 6 months before the pregnancy.

OBJETIVO: Avaliar os efeitos da gravidez em pacientes com lúpus eritematoso sistêmico (LES). MéTODOS: Estudo de coorte retrospectivo. Os dados foram coletados de prontuários de mulheres com LES que engravidaram de janeiro de 2002 a dezembro de 2012 na Universidade Estadual de Campinas, São Paulo, Brasil. Lúpus eritematoso sistêmico e atividade da doença foram definidos segundo o American College of Rheumatology e os critérios do Índice de Atividade da Doença de Lúpus Eritematoso (SLEDAI, na sigla em inglês), respectivamente. As médias, os desvios-padrão (DP), as porcentagens e as correlações foram realizados utilizando o software SAS, versão 9.4 (SAS Institute Inc., Cary, NC, US). RESULTADOS: Obtivemos dados de 69 gestações em 58 mulheres. Durante a gravidez, a reatividade da doença foi observada em 39.2% (n = 27). As manifestações mais comuns foram em pacientes com doença renal prévia, e ocorreram principalmente no terceiro trimestre e no puerpério. Atividade renal ocorreu em 24,6% (n = 17), e atividade grave, em 16% (n = 11). De todos os partos, 75% (n = 48) foram por cesariana. Dois óbitos maternos ocorreram (3%). A prematuridade foi a principal complicação nos recém-nascidos. A taxa de aborto foi de 8,7%. O índice SLEDAI grave durante a gestação foi associado à prematuridade (100%) e à morte perinatal (54%). CONCLUSãO: O resultado materno-fetal é pior no LES quando as mulheres sofrem crise de reativação durante a gravidez. Os melhores desfechos materno-fetais ocorrem quando a doença está em remissão por pelo menos 6 meses anteriores à gestação.

Systemic lupus erythematosus and pregnancy: A single-center observational study of 69 pregnancies / Lúpus eritematoso sistêmico e gravidez: estudo observacional em um único centro com 69 gestações

Abstract Objective To evaluate the effects of pregnancy in systemic lupus erythematosus (SLE) patients. Methods The present article is a retrospective cohort study. Datawere collected from medical records of pregnant women with SLE from January 2002 to December 2012 at Universidade Estadual de Campinas, in the city of Campinas, state of São Paulo, Brazil. Systemic lupus erythematosus and disease activity were defined according to the American College of Rheumatology and the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) criteria respectively. The means, standard deviations (SDs), percentages and correlations were performed using the SAS software, version 9.4 (SAS Institute Inc., Cary, NC, US). Results We obtained data from 69 pregnancies in 58 women. During pregnancy, a new flare was observed in 39.2% (n = 27). The manifestations were most common in patients with prior kidney disease, and mainly occurred during the third quarter and the puerperium. Renal activity occurred in 24.6% (n = 17), and serious activity, in 16% (n = 11). Of all deliveries, 75% (n = 48) were by cesarean section. Twomaternal deaths occurred (3%). Preterm birth was themain complication in the newborns. The abortion rate was 8.7%. Severe SLEDAI during pregnancy was associated with prematurity (100%) and perinatal death (54%). Conclusion Thematernal-fetal outcome is worse in SLE when thewomen experience a flare during pregnancy. The best maternal-fetal outcomes occur when the disease is in remission for at least 6 months before the pregnancy.

Resumo Objetivo Avaliar os efeitos da gravidez em pacientes com lúpus eritematoso sistêmico (LES). Métodos Estudo de coorte retrospectivo. Os dados foram coletados de prontuários de mulheres com LES que engravidaram de janeiro de 2002 a dezembro de 2012 na Universidade Estadual de Campinas, São Paulo, Brasil. Lúpus eritematoso sistêmico e atividade da doença foram definidos segundo o American College of Rheumatology e os critérios doÍndice deAtividadedaDoença de Lúpus Eritematoso (SLEDAI, nasigla eminglês), respectivamente. As médias, os desvios-padrão (DP), as porcentagens e as correlações foram realizados utilizando o software SAS, versão 9.4 (SAS Institute Inc., Cary, NC, US). Resultados Obtivemos dados de 69 gestações em58mulheres. Durante a gravidez, a reatividade da doença foi observada em 39.2% (n = 27). As manifestações mais comuns foram em pacientes com doença renal prévia, e ocorreram principalmente no terceiro trimestre e no puerpério. Atividade renal ocorreu em 24,6% (n = 17), e atividade grave, em 16% (n = 11). De todos os partos, 75% (n = 48) foram por cesariana. Dois óbitos maternos ocorreram (3%). A prematuridade foi a principal complicação nos recém-nascidos. A taxa de aborto foi de 8,7%. O índice SLEDAI grave durante a gestação foi associado à prematuridade (100%) e à morte perinatal (54%). Conclusão O resultado materno-fetal é pior no LES quando as mulheres sofrem crise de reativação durante a gravidez. Os melhores desfechos materno-fetais ocorrem quando a doença está em remissão por pelo menos 6 meses anteriores à gestação.

Brain diffusion tensor MRI in systematic lupus erythematosus: A systematic review.

Diffusion tensor imaging (DTI) maps the brain's microstructure by measuring fractional anisotropy (FA) and mean diffusivity (MD). This systematic review describes brain diffusion tensor Magnetic resonance imaging (MRI) studies in systemic lupus erythematosus (SLE).The literature was reviewed following the PRISMA guidelines and using the terms "lupus", "systemic lupus erythematosus", "SLE", "diffusion tensor imaging", "DTI", "white matter" (WM), "microstructural damage", "tractography", and "fractional anisotropy"; the search included articles published in English from January 2007 to April 2017. The subjects included in the study were selected according to the ACR criteria and included 195 SLE patients with neuropsychiatric manifestation (NPSLE), 299 without neuropsychiatric manifestation (non-NPSLE), and 423 healthy controls (HC). Most studies identified significantly reduced FA and increased MD values in several WM regions of both NPSLE and non-NPSLE patients compared to HC. Subclinical microstructural changes were observed in either regional areas or the entire brain in both the non-NPSLE and NPSLE groups.

Interferon-γ Is Associated with Cerebral Atrophy in Systemic Lupus Erythematosus.

OBJECTIVE: We evaluated the cerebrospinal fluid (CSF) cytokine profile and magnetic resonance imaging (MRI) findings in systemic lupus erythematosus (SLE) patients with central nervous system (CNS) involvement. METHODS: Consecutive SLE patients followed at the rheumatology unit were enrolled into this study. Neurologically asymptomatic controls were matched for age and sex and recruited during myelography. SLE patients were assessed for disease activity (Systemic Lupus Erythematosus Disease Activity Index; SLEDAI) and cumulative damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index; SDI). All subjects underwent MRI scans and blood and CSF withdrawal. Immunoglobulin G (IgG) and albumin were measured by nephelometry and link indexes were calculated according to the literature. Interleukin (IL)-12 p40/p70, interferon (IFN)-γ, tumor necrosis factor (TNF)-α, and IL-10 were measured by enzyme-linked immunosorbent assay. RESULTS: We included 20 SLE patients (18 women, mean age 30.2 ± 9.2 years, range 19-45) with CNS manifestations. Increased IL-12 p40/p70, IFN-γ, TNF-α, and IL-10 CSF levels were observed in SLE patients. Mild pleocytosis was observed in 8 (66%) SLE patients and intrathecal production of IgG was observed in 2 (10%) SLE patients. Three (15%) SLE patients had demyelinating lesions, 5 (25%) patients had cerebral atrophy, and 12 (60%) patients had ischemic lesions on MRI. We observed that the cerebral lesion count was associated with CNS manifestations and SDI scores. We observed a significant cerebral volume reduction in SLE patients compared to controls (p < 0.001). Moreover, a direct correlation between cerebral volume reduction and CSF IFN-γ levels was observed (r = 0.5, p = 0.01). CONCLUSIONS: IL-12 p40/p70, IFN-γ, TNF-α, and IL-10 CSF levels were increased in SLE patients with CNS manifestations, but only IFN-γ was associated with a cerebral volume reduction in SLE, suggesting an immunological basis for global atrophy in SLE.

Myelopathy in systemic lupus erythematosus: clinical, laboratory, radiological and progression findings in a cohort of 1,193 patients.

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.

Myelopathy in systemic lupus erythematosus: clinical, laboratory, radiological and progression findings in a cohort of 1,193 patients / Mielopatia no lúpus eritematoso sistêmico: achados clínicos, laboratoriais, radiológicos e evolutivos em uma coorte de 1. 193 pacientes

Abstract 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.

Resumo Objetivo Descrever características clínicas, laboratoriais, radiológicas e evolutivas de mielopatia no lúpus eritematoso sistêmico (LES). Pacientes e métodos Foi feita análise retrospectiva de uma coorte de 1.193 pacientes com LES (critérios ACR) para identificar os pacientes com mielopatia (ACR neuropsiquiátrico). A atividade de doença foi analisada pelo Índice de Atividade do LES (Sledai) na data do evento e a capacidade funcional pela Escala Expandida do Estado de Incapacidade (EDSS) na última consulta. Resultados Foram identificados 14 (1,2%) pacientes com mielopatia. Todas eram mulheres com média de 30 anos (DP ± 11,5 anos). A mielopatia ocorreu no diagnóstico do LES em quatro (28%) e em nove (64%) havia outro tipo de manifestação neuropsiquiátrica associada. Recorrência do quadro neurológico foi observado em uma (7%) paciente. Atividade de doença foi observada em dois (14%) pacientes. O líquido cefalorraquidiano apresentava pleocitose em sete (53%) pacientes anticorpos antifosfolípides eram positivos em cinco (45%). A ressonância magnética (RM) demonstrou hipersinal em T2 com predomínio do comprometimento longitudinal em seis (86%) pacientes. A maioria foi tratada com corticosteroides e ciclofosfamida endovenosos. Nenhuma paciente teve completa recuperação e quatro (36%) tinham escores altos da EDSS. Óbito foi observado em três (21%) durante episódio de mielopatia, por septicemia durante ou após terapia imunossupressora. Conclusões A mielopatia ocorreu em 14 (1,2%) dos pacientes da nossa coorte e pode ser a primeira manifestação da doença e ocorrer independentemente de atividade sistêmica da doença. Embora rara, é de grande morbimortalidade, pode ser recorrente e a RM é fundamental para o diagnóstico.

Depressive symptoms are associated with tumor necrosis factor alpha in systemic lupus erythematosus.

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.

Mortality in Brazilian community-dwelling older adults: 7 years of follow up in primary care.

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.

Significados do adoecer para pacientes com lúpus eritematoso sistêmico: revisão da literatura / Meanings of the sickening process for patients with systemic lupus erythematosus: a review of the literature

Resumo O lúpus eritematoso sistêmico é uma doença autoimune, que provoca diversas repercussões psicológicas que têm sido estudadas por meio de pesquisas qualitativas. Essas são consideradas relevantes, uma vez que reveladoras da amplitude vivenciada pelos pacientes. Face a essa relevância, este artigo buscou mapear a produção qualitativa na temática, oriunda de estudos de vivências de pacientes adultos de ambos os gêneros e que tivessem usado como instrumento a entrevista semiestruturada e/ou a observação de campo, além do critério de amostragem por saturação na definição do número de participantes de cada estudo. O levantamento foi feito nas bases de dados Pubmed, Lilacs, Psycinfo e Cochrane em busca de produções em língua inglesa e portuguesa publicadas entre janeiro de 2005 e junho de 2012. Os 19 artigos revisados que se ocuparam com pacientes em fase aguda da doença revelaram temas categorizados em oito tópicos que contemplaram o processo vivenciado nas diversas etapas, desde o desencadeamento da doença, passando pelo conhecimento do diagnóstico pela e compreensão das manifestações da doença, até o tratamento medicamentoso e os cuidados gerais, a evolução e o prognóstico. Apontam também a difícil compreensão pelos pacientes do que consiste a chamada fase de remissão, bem como nos revela que se trata de uma fase clínica pouco explorada pelos estudos psicológicos.

Abstract 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.

Serum interleukin-17 levels are associated with nephritis in childhood-onset systemic lupus erythematosus.

OBJECTIVES: To determine the serum interleukin-17 (IL-17) levels in childhood-onset systemic lupus erythematosus patients and to evaluate the association between IL-17 and clinical manifestations, disease activity, laboratory findings and treatment. METHODS: We included 67 consecutive childhood-onset systemic lupus erythematosus patients [61 women; median age 18 years (range 11-31)], 55 first-degree relatives [50 women; median age 40 years (range 29-52)] and 47 age- and sex-matched healthy controls [42 women; median age 19 years (range 6-30)]. The childhood-onset systemic lupus erythematosus patients were assessed for clinical and laboratory systemic lupus erythematosus manifestations, disease activity [Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)], cumulative damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology (ACR) Damage Index] and current drug use. Serum IL-17 levels were measured by an enzyme-linked immunosorbent assay using commercial kits. RESULTS: The median serum IL-17 level was 36.3 (range 17.36-105.92) pg/mL in childhood-onset systemic lupus erythematosus patients and 29.47 (15.16-62.17) pg/mL in healthy controls (p=0.009). We observed an association between serum IL-17 levels and active nephritis (p=0.01) and migraines (p=0.03). Serum IL-17 levels were not associated with disease activity (p=0.32), cumulative damage (p=0.34), or medication use (p=0.63). CONCLUSION: IL-17 is increased in childhood-onset systemic lupus erythematosus and may play a role in the pathogenesis of neuropsychiatric and renal manifestations. Longitudinal studies are necessary to determine the role of IL-17 in childhood-onset systemic lupus erythematosus.

Serum interleukin-17 levels are associated with nephritis in childhood-onset systemic lupus erythematosus

OBJECTIVES: To determine the serum interleukin-17 (IL-17) levels in childhood-onset systemic lupus erythematosus patients and to evaluate the association between IL-17 and clinical manifestations, disease activity, laboratory findings and treatment. METHODS: We included 67 consecutive childhood-onset systemic lupus erythematosus patients [61 women; median age 18 years (range 11-31)], 55 first-degree relatives [50 women; median age 40 years (range 29-52)] and 47 age- and sex-matched healthy controls [42 women; median age 19 years (range 6-30)]. The childhood-onset systemic lupus erythematosus patients were assessed for clinical and laboratory systemic lupus erythematosus manifestations, disease activity [Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)], cumulative damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology (ACR) Damage Index] and current drug use. Serum IL-17 levels were measured by an enzyme-linked immunosorbent assay using commercial kits. RESULTS: The median serum IL-17 level was 36.3 (range 17.36-105.92) pg/mL in childhood-onset systemic lupus erythematosus patients and 29.47 (15.16-62.17) pg/mL in healthy controls (p=0.009). We observed an association between serum IL-17 levels and active nephritis (p=0.01) and migraines (p=0.03). Serum IL-17 levels were not associated with disease activity (p=0.32), cumulative damage (p=0.34), or medication use (p=0.63). CONCLUSION: IL-17 is increased in childhood-onset systemic lupus erythematosus and may play a role in the pathogenesis of neuropsychiatric and renal manifestations. Longitudinal studies are necessary to determine the role of IL-17 in childhood-onset systemic lupus erythematosus. .

Consenso da Sociedade Brasileira de Reumatologia para o diagnóstico, manejo e tratamento da nefrite lúpica / Consensus of the Brazilian Society of Rheumatology for the diagnosis, management and treatment of lupus nephritis

Objetivo Elaborar recomendações para o diagnóstico, manejo e tratamento da nefrite lúpica no Brasil. Método Revisão extensa da literatura com seleção dos artigos com base na força de evidência científica e opinião dos membros da Comissão de Lúpus Eritematoso Sistêmico da Sociedade Brasileira de Reumatologia. Resultados e conclusões 1) A biópsia renal deve ser feita sempre que possível e houver indicação e quando não for possível, o tratamento deve ser orientado com base na inferência da clase histológica. 2) Devem ser implementados medidas e cuidados idealmente antes do início do tratamento, com ênfase na atenção ao risco de infecção. 3) Devem-se compartilhar riscos e benefícios do tratamento com pacientes e familiares. 4) O uso da hidroxicloroquina (preferencialmente) ou difosfato de cloroquina é recomendado para todos os pacientes (exceto contraindicação) durante as fases de indução e manutenção. 5) A avaliação da eficácia do tratamento deve ser feita com critérios objetivos de resposta (remissão completa/remissão parcial/refratariedade). 6) Os IECA e/ou BRA são recomendados como antiproteinúricos para todos os pacientes (exceto contraindicação). 7) A identificação de sinais clínicos e/ou laboratoriais sugestivos de GN laboratoriais sugestivos de glomerulonefrite proliferativa ou membranosa deve indicar início imediato de terapia específica incluindo corticosteroides e agente imunossupressor, mesmo que não seja possível comprovação histológica. 8) O tempo de uso dos imunossupressores deve ser no mínimo de 36 meses, mas eles podem ser mantidos por períodos mais longos. A sua suspensão só deve ser feita quando o paciente atingir e mantiver remissão completa sustentada. 9) Deve-se considerar nefrite lúpica refratária quando a remissão completa ou parcial não for alcançada após 12 meses de tratamento adequado, quando uma nova biópsia renal deve ser considerada para auxiliar na identificação da causa da refratariedade e decisão terapêutica. .

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) Angiotensin-converting enzyme inhibitors and/or angiotensin receptor blockers 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 corticosteroids 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 could 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 ...

[Consensus of the Brazilian Society of Rheumatology for the diagnosis, management and treatment of lupus nephritis]. / Consenso da Sociedade Brasileira de Reumatologia para o diagnóstico, manejo e tratamento da nefrite lúpica.

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.

Meanings of the sickening process for patients with systemic lupus erythematosus: a review of the literature.

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.

Evaluation of respiratory impairment in patients with systemic lupus erythematosus with the six-minute walk test.

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.