ANCA in patients with systemic lupus erythematosus. A cross sectional study in Brazilian patients and review of literature.

BACKGROUND: Antineutrophil cytoplasmatic antibodies (ANCA) have been detected in patients with systemic lupus erythematosus (SLE). In this study, we investigated the presence of ANCA in a sample of Brazilian SLE patients and its possible associations with clinical and serological outcomes. Additionally, we reviewed the literature of on ANCA in SLE. RESULTS: The presence of ANCA was detected in 130 patients using indirect immunofluorescence (IIF). The test was positive in 29.9% of the cases (17.6% pANCA and 11.5% cANCA). Male sex and peripheral vasculitis were more prevalent in the ANCA-positive sample. cANCA was associated with lupus anticoagulant and pANCA had a positive association with peripheral vasculitis and a negative association with anti- SSB/La antibodies. In the 22 studies included in the literature review, a wide range of ANCA positivity was found (13% to 81.1% by IIF and 0 to 22.2% by ELISA). ANCA was associated with renal damage in the Asian population. Although other associations have been found in isolated studies, they were not consistently reported. CONCLUSIONS: The ANCA prevalence found in this Brazilian sample was within the range reported in the literature and these autoantibodies were more frequent in males and in patients with vasculitis. The literature showed controversial results on the association between ANCA and SLE disease activity or clinical characteristics.

CHARLSON'S COORDINITY INDEX IN SYSTEMIC LUPUS ERYTHEMATOSUS IN BRAZILIAN PATIENTS / ÍNDICE DE COMORBIDADES DE CHARLSON NO LÚPUS ERITEMATOSO SISTÊMICO EM PACIENTES BRASILEIRAS

Introduction: The Charlson comorbidities index (CCI) assesses a person's chances of survival over the next 10 years. In systemic lupus erythematosus (SLE), multiple comorbidities and complications affect patient survival. Objetive: Analize the variables that influence the CCI of a group of females with SLE. Methods: Retrospective study of medical records of 100 lupus patients for CCI, clinical, epidemiological and serological variables. Results: No epidemiological variable interfered in CCI. Regarding clinical manifestations, patients with glomerulonephritis had a worse CCI than those without (p<0.0001) and those with central nervous system manifestations had a tendency to worse CCI (p=0.09). Patients with anti-Ro antibodies (p=0.02) and rheumatoid factor or RF (p=0.002) were associated with a lower CCI. Conclusions: The presence of glomerulonephritis is associated with lower survival and of the anti-Ro and RF antibodies with longer survival in SLE.

Introdução : O índice de comorbidades de Charlson (ICC) avalia as chances de sobrevivência de uma pessoa nos próximos 10 anos. No lúpus eritematoso sistêmico (LES) múltiplas comorbidades e complicações afetam a sobrevida. Objetivo : Verificar as variáveis ​​que influenciam no ICC de um grupo de mulheres com LES. Métodos : Estudo retrospectivo de 100 pacientes lúpicas para o ICC, variáveis ​​clínicas, epidemiológicas e sorológicas. Resultados : Nenhuma variável epidemiológica interferiu no ICC. Quanto à clínica, pacientes com glomerulonefrite tiveram pior ICC do que os sem (p<0,0001) e os com manifestações de sistema nervoso central tiveram tendência para pior ICC (p=0,09). Portadores de anticorpos anti-Ro (p=0,02) e fator reumatoide (FR; p=0,002) se associaram com ICC menor. Conclusões : A presença de glomerulonefrite se associa com menor sobrevida, e a dos anticorpos anti-Ro e FR com maior sobrevida no LES.

Cognitive dysfunction in patients with systemic lupus erythematosus. A cross-sectional study in a Brazilian sample.

BACKGROUND: Cognitive dysfunction (CD) is frequently found in patients with systemic lupus erythematosus (SLE) and contributes to impairment in the patient's quality of life. AIM: To study CD in a sample of patients and the possible associations with cumulative damage, disease activity, clinical/serological profile, and cumulative glucocorticoid dose. METHODS: Included in this study are 103 patients with SLE and 95 controls for cognitive performance through MoCA (Montreal Cognitive Assessment) and MMSE (Mini-Mental State Examination). Disease activity was measured by SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) and cumulative organ damage by SLICC/ACR/DI (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index). CES-D (Center for Epidemiological Studies-Depression) scale was used to evaluate depression. Data on clinical and serological profile, treatment, and cumulative glucocorticoid dose were also collected. RESULTS: Patients with SLE had worse performance in the MoCA (p = 0.009) and MMSE (p = 0.01) than controls. The MoCA results showed that visuospatial and abstraction domains (p = 0.03 and p = 0.002, respectively) were impaired, and the MMSE results showed that language and spatial orientation were reduced (p = 0.002 and p = 0.01, respectively) when compared to controls. Both questionnaires (MoCA with r = -0.29 and MMSE r = -0.21) correlated negatively with SLICC/ACR/DI and the MoCA with SLEDAI (r = -0.22). No associations were found with cumulative glucocorticoid dose, degree of depression, and clinical or serological profile. CONCLUSIONS: According to the MoCA, visuospatial cognition and abstraction were impaired, and according to the MMSE, spatial orientation and language were impaired in patients with SLE. The CD was correlated with cumulative damage and disease activity. These findings demonstrate that both disease-activity and disease-injury associated CD is found broadly in SLE patients in a Brazilian population, supporting the prior reports of CD in other regional SLE populations.

Índice de comorbidades de Charlson no lúpus eritematoso sistêmico em pacientes brasileiras / Charlson's coordinity index in systemic lupus erythematosus in brazilian patients

Introdução: O índice de comorbidades de Charlson (ICC) avalia as chances de sobrevida de uma pessoa nos próximos 10 anos. No lúpus eritematoso sistêmico (LES) múltiplas comorbidades e complicações afetam a sobrevida. Objetivo: Verificar as variáveis que influem no ICC de um grupo de mulheres com LES. Métodos: Estudo retrospectivo de 100 pacientes lúpicas para o ICC, variáveis clínicas, epidemiológicas e sorológicas. Resultados: Nenhuma variável epidemiológica interferiu no ICC. Quanto à clínica, pacientes com glomerulonefrite tiveram pior ICC do que os sem (p<0,0001) e os com manifestações de sistema nervoso central tiveram tendência para pior ICC (p=0,09). Portadores de anticorpos anti-Ro (p=0,02) e fator reumatoide (FR; p=0,002) se associaram com ICC menor. Conclusões: A presença de glomerulonefrite se associa com menor sobrevida, e a dos anticorpos anti-Ro e FR com maior sobrevida no LES.

Introduction: The Charlson comorbidities index (CCI) assesses a person's chances of survival over the next 10 years. In systemic lupus erythematosus (SLE), multiple comorbidities and complications affect patient survival. Objetive: Analize the variables that influence the CCI of a group of females with SLE. Methods: Retrospective study of medical records of 100 lupus patients for CCI, clinical, epidemiological and serological variables. Results: No epidemiological variable interfered in CCI. Regarding clinical manifestations, patients with glomerulonephritis had a worse CCI than those without (p<0.0001) and those with central nervous system manifestations had a tendency to worse CCI (p=0.09). Patients with anti-Ro antibodies (p=0.02) and rheumatoid factor or RF (p=0.002) were associated with a lower CCI. Conclusions: The presence of glomerulonephritis is associated with lower survival and of the anti-Ro and RF antibodies with longer survival in SLE.

Serum immunoglobulin a deficiency and autoimmune comorbidities: a crossectional study in 281 patients with systemic lupus erythematosus.

OBJECTIVE To study the profile of associated autoimmune diseases in a series of patients with systemic lupus erythematosus (SLE) and see if such associations are linked to IgA deficiency. METHODS Two hundred eighty-one patients with SLE were studied for Ig A levels by nephelometry. Levels equal to or under 0.05g/dL were considered as IgA deficiency. Epidemiological and clinical data, including the presence of associated autoimmune diseases, were extracted from the patient's charts. RESULTS Ig A deficiency was found in 6% of the patients. In 30.2% of SLE patients, there was at least one more autoimmune disease; Hashimoto thyroiditis and Sjögren's syndrome were the most common. No association between the occurrence of associated autoimmune disease with IgA deficiency was found. CONCLUSIONS There is a high prevalence of autoimmune diseases associated with SLE. IgA deficiency does not affect the presence of these associations.

Serum immunoglobulin a deficiency and autoimmune comorbidities: a crossectional study in 281 patients with systemic lupus erythematosus

SUMMARY OBJECTIVE To study the profile of associated autoimmune diseases in a series of patients with systemic lupus erythematosus (SLE) and see if such associations are linked to IgA deficiency. METHODS Two hundred eighty-one patients with SLE were studied for Ig A levels by nephelometry. Levels equal to or under 0.05g/dL were considered as IgA deficiency. Epidemiological and clinical data, including the presence of associated autoimmune diseases, were extracted from the patient's charts. RESULTS Ig A deficiency was found in 6% of the patients. In 30.2% of SLE patients, there was at least one more autoimmune disease; Hashimoto thyroiditis and Sjögren's syndrome were the most common. No association between the occurrence of associated autoimmune disease with IgA deficiency was found. CONCLUSIONS There is a high prevalence of autoimmune diseases associated with SLE. IgA deficiency does not affect the presence of these associations.

RESUMO OBJETIVO Estudar o perfil de doenças autoimunes associadas em uma série de pacientes com lúpus eritematoso sistêmico (LES) e verificar se tais associações estão ligadas à deficiência de imunoglobulina (Ig) A. MÉTODOS Foram estudados 281 pacientes com LES para os níveis de IgA por nefelometria. Níveis iguais ou menores que 0,05 g/dL foram considerados como deficiência dessa imunoglobulina. Dados epidemiológicos e clínicos, incluindo a presença de doenças autoimunes associadas, foram extraídos dos prontuários dos pacientes. RESULTADOS A deficiência de IgA foi encontrada em 6% dos pacientes. Em 30,2% dos pacientes com LES encontrou-se a presença de, pelo menos, mais uma doença autoimune. Tireoidite de Hashimoto e síndrome de Sjögren foram as mais comuns. Não foi possível ligar a ocorrência de uma doença autoimune associada ao LES com deficiência de IgA. CONCLUSÕES Existe uma alta prevalência de doenças autoimunes associadas ao LES. A deficiência de IgA não afeta a presença dessas associações.

Comparison of lupus patients with early and late onset nephritis: a study in 71 patients from a single referral center.

BACKGROUND: Nephritis occurs frequently in systemic lupus erythematosus (SLE) and may worsen disease morbidity and mortality. Knowing all characteristics of this manifestation helps to a prompt recognition and treatment. AIM: To compare the differences in clinical data, serological profile and treatment response of nephritis of early and late onset. METHODS: Retrospective study of 71 SLE patients with biopsy proven nephritis divided in early nephritis group (diagnosis of nephritis in the first 5 years of the disease) and late nephritis (diagnosis of nephritis after 5 years). Epidemiological, serological, clinical and treatment data were collected from charts and compared. RESULTS: In this sample, 70. 4% had early onset nephritis and 29.6% had late onset. No differences were noted in epidemiological, clinical, serological profile, SLICC and SLEDAI, except that late onset nephritis patients were older at nephritis diagnosis (p = 0.01). Regarding renal biopsy classification, C3 and C4 levels, serum creatinine, 24 h proteinuria and response rate to treatment the two groups were similar (p = NS). Patients with early onset had lower levels of hemoglobin at nephritis onset than those of late onset (p = 0.02). CONCLUSIONS: Most of SLE patients had nephritis in the first 5 years of disease. No major differences were noted when disease profile or treatment outcome of early and late onset nephritis were compared.

Comparison of lupus patients with early and late onset nephritis: a study in 71 patients from a single referral center

Abstract Background: Nephritis occurs frequently in systemic lupus erythematosus (SLE) and may worsen disease morbidity and mortality. Knowing all characteristics of this manifestation helps to a prompt recognition and treatment. Aim: To compare the differences in clinical data, serological profile and treatment response of nephritis of early and late onset. Methods: Retrospective study of 71 SLE patients with biopsy proven nephritis divided in early nephritis group (diagnosis of nephritis in the first 5 years of the disease) and late nephritis (diagnosis of nephritis after 5 years). Epidemiological, serological, clinical and treatment data were collected from charts and compared. Results: In this sample, 70. 4% had early onset nephritis and 29.6% had late onset. No differences were noted in epidemiological, clinical, serological profile, SLICC and SLEDAI, except that late onset nephritis patients were older at nephritis diagnosis (p = 0.01). Regarding renal biopsy classification, C3 and C4 levels, serum creatinine, 24 h proteinuria and response rate to treatment the two groups were similar (p = NS). Patients with early onset had lower levels of hemoglobin at nephritis onset than those of late onset (p = 0.02). Conclusions: Most of SLE patients had nephritis in the first 5 years of disease. No major differences were noted when disease profile or treatment outcome of early and late onset nephritis were compared.(AU)

The Brazillian version of the hand mobility in scleroderma (HAMIS) test: translation and validation.

BACKGROUND: The Hand Mobility in Scleroderma (HAMIS) test was created to measure the degree of dysfunction of hand movements imposed by systemic sclerosis (SSc). The modified version (mHAMIS), with 4 of the 9 original items, was developed later. The goal of the present study was to translate and validate HAMIS and mHAMIS into Brazilian Portuguese and culture. METHODS: After direct and reverse translation and comprehension test in 10 SSc patients, HAMIS-Br was applied to another 32 patients with SSc. To evaluate internal consistency, intraobserver and interobserver agreement, and intraobserver and interobserver reliability, we used respectively the Cronbach's α coefficient, kappa concordance and intraclass correlation (ICC). The correlation between HAMIS-Br and mHAMIS-Br was evaluated and a factorial analysis was performed. RESULTS: HAMIS-Br showed excellent internal consistency (Cronbach's α = 0.997), good intraobserver agreement (kappa between 0.78 [95%CI =0.57-0.99] and 1) and intraobserver and interobserver reliability (ICC = 0.993, 95% CI = 0.973-0.993 and ICC = 0.994, 95% CI = 0.987-0.997, respectively). The mHAMIS-Br presented similar results and excellent correlation with HAMIS-Br (r = 0.923). The factorial analysis extracted three groups of questions that explain 84.4% of the total variance, and that can be understood through the influence of certain movements in the interpretation of others: [1] questions whose interpretation is influenced by the extension of the fingers, [2] questions whose interpretation is influenced by flexion of the fingers, [3] volar flexion of the fingers, with similar correlation with both other factors. CONCLUSIONS: HAMIS-Br and mHAMIS-Br showed good agreement, intraobserver and interobserver reliability, and internal validity. It is necessary to be attentive to the influence of certain limitations of movements in the interpretation of others.

Sarcoidose e sacroiliíte: relato de caso e revisão da literatura / Sarcoidosis and sacroiliitis: case report and literature review

A sarcoidose é uma doença granulomatosa sistêmica, de etiologia ainda desconhecida. Essa doença pode acometer qualquer órgão, mas afeta principalmente pulmão e gânglios linfáticos intratorácicos. Em apenas 10% dos casos existe envolvimento extra torácico. O envolvimento articular é raro e pode-se dividir em agudo e crônico. As articulações mais afetadas na sarcoidose são as dos joelhos, punho, cotovelo e articulações das mãos e pés. Neste estudo iremos descrever um caso de um paciente com sarcoidose envolvendo a articulação sacriilíaca. O acometimento dessa região é bem raro e existem poucos casos relatados na literatura

Sarcoidosis is a systemic granulomatous disease of unknown etiology. This disease can affect any organ, but prefers lung and intra thoracic lymph nodes. In just 10% of cases there is extra thoracic involvement. Joint involvement is rare and can be divided into acute and chronic involvment. The most affected joints are the knees, wrist, elbow, and joints of the hands and feet. Herein we describe a case of a patient with sarcoidosis involving the sacriiliac joint. The involvement of this region is very rare and there are few cases reported in the literature

Sarcoidose e sacroiliíte: relato de caso e revisão da literatura / Sarcoidosis and sacroiliitis: case report and literature review

A sarcoidose é uma doença granulomatosa sistêmica, de etiologia ainda desconhecida. Essa doença pode acometer qualquer órgão, mas afeta principalmente pulmão e gânglios linfáticos intratorácicos. Em apenas 10% dos casos existe envolvimento extra torácico. O envolvimento articular é raro e pode-se dividir em agudo e crônico. As articulações mais afetadas na sarcoidose são as dos joelhos, punho, cotovelo e articulações das mãos e pés. Neste estudo iremos descrever um caso de um paciente com sarcoidose envolvendo a articulação sacriilíaca. O acometimento dessa região é bem raro e existem poucos casos relatados na literatura

Sarcoidosis is a systemic granulomatous disease of unknown etiology. This disease can affect any organ, but prefers lung and intra thoracic lymph nodes. In just 10% of cases there is extra thoracic involvement. Joint involvement is rare and can be divided into acute and chronic involvment. The most affected joints are the knees, wrist, elbow, and joints of the hands and feet. Herein we describe a case of a patient with sarcoidosis involving the sacriiliac joint. The involvement of this region is very rare and there are few cases reported in the literature

The Brazillian version of the hand mobility in scleroderma (HAMIS) test: translation and validation

Abstract Background: The Hand Mobility in Scleroderma (HAMIS) test was created to measure the degree of dysfunction of hand movements imposed by systemic sclerosis (SSc). The modified version (mHAMIS), with 4 of the 9 original items, was developed later. The goal of the present study was to translate and validate HAMIS and mHAMIS into Brazilian Portuguese and culture. Methods: After direct and reverse translation and comprehension test in 10 SSc patients, HAMIS-Br was applied to another 32 patients with SSc. To evaluate internal consistency, intraobserver and interobserver agreement, and intraobserver and interobserver reliability, we used respectively the Cronbach's α coefficient, kappa concordance and intraclass correlation (ICC). The correlation between HAMIS-Br and mHAMIS-Br was evaluated and a factorial analysis was performed. Results: HAMIS-Br showed excellent internal consistency (Cronbach's α = 0.997), good intraobserver agreement (kappa between 0.78 [95%CI =0.57-0.99] and 1) and intraobserver and interobserver reliability (ICC = 0.993, 95% CI = 0.973-0.993 and ICC = 0.994, 95% CI = 0.987-0.997, respectively). The mHAMIS-Br presented similar results and excellent correlation with HAMIS-Br (r = 0.923). The factorial analysis extracted three groups of questions that explain 84.4% of the total variance, and that can be understood through the influence of certain movements in the interpretation of others: [1] questions whose interpretation is influenced by the extension of the fingers, [2] questions whose interpretation is influenced by flexion of the fingers, [3] volar flexion of the fingers, with similar correlation with both other factors. Conclusions: HAMIS-Br and mHAMIS-Br showed good agreement, intraobserver and interobserver reliability, and internal validity. It is necessary to be attentive to the influence of certain limitations of movements in the interpretation of others.

Direct antiglobulin (Coombs) test in systemic lupus erythematosus patients.

The objective of the study is to study the positivity of Coombs test or direct antiglobulin test (DAT) in systemic lupus erythematosus (SLE) patients and its relationship with disease's clinical and serological profile. Retrospective study of 373 SLE patients seen at single Rheumatology Unit. Epidemiological data (age, gender, age at disease onset, auto declared ethnic background and tobacco use), clinical (malar rash, photosensitivity, oral ulcers, discoid lesions, serositis, glomerulonephritis, convulsions, psychosis, hemolytic anemia, leukopenia, lymphocytopenia and arthritis), and serological profile (anti ds DNA, anti Ro/SS-A; anti La/SS-B, Anti RNP, Anti Sm, aCl (anticardiolipin) IgG, aCl Ig M, LA or lupus anticoagulant, rheumatoid factor and direct Coombs) were collected. Patients with a positive DAT were compared with the negatives. DAT was positive in 12.8% of patients and 54.3% of them had hemolytic anemia. In univariate analysis, a positive DAT was associated with hemolytic anemia (p < 0.0001), secondary antiphospholipid antibody syndrome (p = 0.02), anticardiolipin IgG (p < 0.0001), lupus anticoagulant (p = 0.05), positive VDRL (p = 0.004), anti-RNP (p < 0.0001), anti-Sm (p = 0.002), and anti-La (p = 0.02). Logistic regression revealed that hemolytic anemia, anti-RNP and anti-LA were independently associated with positive DAT. DAT was positive in 12.8% of SLE studied sample and 54.3% of them had hemolytic anemia. This test was independently associated with hemolytic anemia, anti-RNP and anti-La antibodies.