Comparison among ACR1997, SLICC and the new EULAR/ACR classification criteria in childhood-onset systemic lupus erythematosus

Abstract Background: To date there are no specific classification criteria for childhood-onset systemic lupus erythematosus (cSLE). This study aims to compare the performance among the American College of Rheumatology (ACR) 1997, the Systemic Lupus International Collaborating Clinics criteria (SLICC) and the new European League Against Rheumatism (EULAR)/ACR criteria, in a cSLE cohort. Methods: We conducted a medical chart review study of cSLE cases and controls with defined rheumatic diseases, both ANA positive, to establish each ACR1997, SLICC and EULAR/ACR criterion fulfilled, at first visit and 1-year-follow-up. Results: Study population included 122 cSLE cases and 89 controls. At first visit, SLICC criteria had higher sensitivity than ACR 1997 (89.3% versus 70.5%, p < 0.001), but similar specificity (80.9% versus 83.2%, p = 0.791), however performance was not statistically different at 1-year-follow-up. SLICC better scored in specificity compared to EULAR/ACR score ≥ 10 at first visit (80.9% versus 67.4%, p = 0.008) and at 1-year (76.4% versus 58.4%, p = 0.001), although sensitivities were similar. EULAR/ACR criteria score ≥ 10 exhibited higher sensitivity than ACR 1997 (87.7% versus 70.5%, p < 0.001) at first visit, but comparable at 1-year, whereas specificity was lower at first visit (67.4% versus 83.2%, p = 0.004) and 1-year (58.4% versus 76.4%, p = 0.002). A EULAR/ACR score ≥ 13 against a score ≥ 10, resulted in higher specificity, positive predictive value, and cut-off point accuracy. Compared to SLICC, a EULAR/ACR score ≥ 13 resulted in lower sensitivity at first visit (76.2% versus 89.3%, p < 0.001) and 1-year (91% versus 97.5%, p = 0.008), but similar specificities at both assessments. When compared to ACR 1997, a EULAR/ACR total score ≥ 13, resulted in no differences in sensitivity and specificity at both observation periods. Conclusions: In this cSLE population, SLICC criteria better scored at first visit and 1-year-follow-up. The adoption of a EULAR/ACR total score ≥ 13 in this study, against the initially proposed ≥10 score, was most appropriate to classify cSLE. Further studies are necessary to address if SLICC criteria might allow fulfillment of cSLE classification earlier in disease course and may be more inclusive of cSLE subjects for clinical studies.

Relevância do estado de hidratação na interpretação de parâmetros nutricionais em diálise peritoneal / Relevance of hydration status on the interpretation of nutritional parameters in peritoneal dialysis

OBJETIVO: Identificar determinantes do estado de hidratação de pacientes em diálise peritoneal crônica, bem como investigar os efeitos da sobrecarga líquida sobre o estado nutricional. MÉTODOS: Foi feito estudo transversal, realizado em 2006, avaliando 27 pacientes em diálise peritoneal crônica, acompanhados no Hospital das Clínicas da Faculdade de Medicina de Botucatu (SP), quanto a parâmetros clínicos, dialíticos, laboratoriais, antropométricos e de bioimpedância elétrica. Para avaliar a influência de parâmetros sobre o estado de hidratação empregou-se modelo de regressão linear múltipla. A amostra foi estratificada quanto ao estado de hidratação pela relação entre água extracelular e água corporal total (0,47 para homens e 0,52 para mulheres), parâmetros obtidos por meio de bioimpedância elétrica. Comparações foram realizadas por análise de covariância, Mann-Whitney, Qui-quadrado ou teste exato de Fisher. Considerou-se significância estatística quando p≤0,05. RESULTADOS: Pacientes com maior volume urinário e em modalidade dialítica automatizada apresentaram melhor estado de hidratação. Pacientes com maior sobrecarga líquida, comparados àqueles com menor sobrecarga, apresentaram menor ângulo de fase (M=4,2, DP=0,9 vs M=5,7, DP=0,7º; p=0,006), menor albumina (M=3,06, DP=0,46 vs M=3,55, DP=0,52g/dL; p=0,05) e maior por cento prega cutânea tricipital (M=75,3, DP=36,9 vs M=92,1, DP=56,9 por cento; p=0,058), sem outras evidências antropométricas. CONCLUSÃO: Pode-se sugerir que os níveis reduzidos de albumina e ângulo de fase nos pacientes com maior sobrecarga líquida não estiveram relacionados a pior estado nutricional. Para o diagnóstico nutricional em vigência de sobrecarga líquida, deve-se considerar o conjunto de variáveis obtidas por diversos métodos, buscando relacioná-las e interpretá-las de maneira abrangente, possibilitando um diagnóstico nutricional fidedigno.

OBJECTIVE: This study identified determinants of the hydration status of chronic peritoneal dialysis patients and investigated the effects of fluid overload on their nutritional status. METHODS: A cross-sectional study was conducted in 2006 to evaluate 27 chronic peritoneal dialysis patients from the Dialysis Center of the Medical School Hospital of Botucatu (SP), considering clinical, dialytic, laboratory, anthropometric and bioimpedance parameters. A linear multiple regression model was used to evaluate the influence of these parameters on hydration status. The sample was stratified according to hydration status, given by the ratio between extracellular water and total body water (0.47 for males and 0.52 for females), obtained by bioelectrical impedance. Analysis of covariance, Mann-Whitney test, chi-square test, and Fisher's exact test were used for making comparisons. The significance level was set at 5 percent (p≤0.05). RESULTS: Patients with greater urine volume and receiving automatic dialysis presented better hydration status. Patients with higher fluid overload, compared with those with lower overload, presented lower phase angle (M=4.2, SD=0.9 vs. M=5.7, SD=0.7º; p=0.006), lower albumin levels (M=3.06, SD=0.46 vs. M=3.55, SD=0.52g/dL; p=0.05), and higher percentage of triceps skinfold thickness (M=75.3, SD=36.9 vs. M= 92.1, SD=56.9; p=0.058). No other anthropometric differences were observed. CONCLUSION: Low levels of albumin and phase angle in patients with higher fluid overload were not related to worse nutritional status. This result suggests that one must consider the set of variables obtained by many methods and relate and interpret them comprehensively in order to obtain a reliable nutritional diagnosis of patients with fluid overload.

The extracellular matrix of the lung and its role in edema formation

The extracellular matrix is composed of a three-dimensional fiber mesh filled with different macromolecules such as: collagen (mainly type I and III), elastin, glycosaminoglycans, and proteoglycans. In the lung, the extracellular matrix has several functions which provide: 1) mechanical tensile and compressive strength and elasticity, 2) low mechanical tissue compliance contributing to the maintenance of normal interstitial fluid dynamics, 3) low resistive pathway for an effective gas exchange, d) control of cell behavior by the binding of growth factors, chemokines, cytokines and the interaction with cell-surface receptors, and e) tissue repair and remodeling. Fragmentation and disorganization of extracellular matrix components comprises the protective role of the extracellular matrix, leading to interstitial and eventually severe lung edema. Thus, once conditions of increased microvascular filtration are established, matrix remodeling proceeds fairly rapidly due to the activation of proteases. Conversely, a massive matrix deposition of collagen fiber decreases interstitial compliance and therefore makes the tissue safety factor stronger. As a result, changes in lung extracellular matrix significantly affect edema formation and distribution in the lung.

A matriz extracelular é um aglomerado tridimensional demacromoléculas composta por: fibras colágenas (principalmente, tipos I e III), elastina, glicosaminoglicanos e proteoglicanos. No pulmão, a matriz extracelular tem várias funções, tais como: 1) promover estresse tensil e elasticidade tecidual, 2) contribuir para a manutenção da dinâmica de fluidos no interstício, 3) propiciar efetiva troca gasosa, 4) controlar a função celular através de sua ligação com fatores de crescimento, quimiocinas, citocinas e interação com receptores de superfície, e 5) remodelamento e reparo tecidual. A fragmentação e a desorganização da matriz extracelular pode acarretar edema intersticial e, eventualmente, edema alveolar grave. Logo, quando há aumento da filtração microvascular ocorre rápido remodelamento da matriz por ativação de proteases. Destarte, a deposição de fibras colágenas reduz a complacência intersticial limitando o edema. Em conclusão, modificações na matriz extracelular podem afetar a formação e distribuição do edema no pulmão.