Systemic lupus erythematosus diagnosed during hospitalization: Greater baseline disease activity, short-term damage, and death / Lupus eritematoso sistémico diagnosticado durante una internación: mayor actividad basal de la enfermedad, daño y muerte a corto plazo

ABSTRACT Objectives: To analyze initial and follow-up features of patients with systemic lupus erythematosus (SLE) diagnosed during hospitalization. Methods: Retrospective analysis of medical records: two groups were studied, a) SLE diagnosed during hospitalization (SLEin), b) SLE diagnosed on an outpatient basis (SLEout). Results: 123 patients were assessed, 87% female, mean age at diagnosis was 34 years and 45 (37%) of them were SLEin. Patients in the SLEin group had a median of 144 days from the onset of symptoms to diagnosis of SLE vs. 287 days in the SLEout group (p = 0.04). Initially, SLEin had an average SLEDAI of 10 vs. 8 in SLEout (p = 0.004) and anti-dsDNA was positive in 71% vs. 53% in SLEout (p = 0.07). Within the first 6 months, the average cumulative glucocorticoid dose was 6493 mg in SLEin patients vs. 3563 mg in SLEout (p < 0.001) and immunosuppressant usage was higher in SLEin: 62% vs. 26% in SLEout (p<0.001). Within the first year, SLEin's kidney biopsies showed lupus nephritis III or IV in 31% vs. 12% in SLEout (p = 0.003, log-rank test). Within the first 2 years, 6 SLEin patients died vs. 1 SLEout patient (p = 0.02) and SLEin patients had more damage as measured by SLICC/ACR Damage Index (median 0, range 25%-75% 0-1 vs. median 0, range 25%-75% 0-0 in SLEout; p = 0.04). Conclusions: SLEin are initially more active, require higher doses of glucocorticoids and immunosuppressants, have more significant kidney involvement, and present more damage and greater mortality in the short term.

RESUMEN Objetivo: Analizar las características de los pacientes con lupus eritematoso sistêmico (LES) diagnosticados durante una hospitalización. Métodos: Análisis retrospectivo de historias clínicas. Se estudiaron dos grupos: a) LES diagnosticado durante la hospitalización (SLEin) y b) LES diagnosticado de forma ambulatoria (SLEout). Resultados: Se evaluaron 123 pacientes (87% mujeres); edad promedio al diagnóstico 34 arios; el 37% de ellos era SLEin. Los pacientes del grupo SLEin tuvieron una mediana de 144 días desde el inicio de los síntomas hasta el diagnóstico, vs. 287 días en SLEout (p = 0,04). Inicialmente, los pacientes SLEin tenían un SLEDAI promedio de 10, vs. 8 en SLEout (p = 0,004) y anti-dsDNA positivo en el 71%, vs. el 53% en SLEout (p = 0,07). A los 6 meses, la dosis acumulada de glucocorticoides (promedio) fue de 6.493 mg en SLEin vs. 3.563 mg en SLEout (p<0,001), y el uso de inmunosupresores fue mayor en SLEin: 62% vs. 26% en SLEout (p< 0,001). Al año se halló nefritis lúpica clase III o IV en el 31% de SLEin vs. el 12% en SLEout (Log Rank Test: p = 0,003). A los 2 años, 6 pacientes de SLEin murieron, vs. un paciente de SLEout (p = 0,02). Los pacientes con SLEin tuvieron más daño (índice de daño SLICC/ACR: mediana 0, rango 25-75%: 0-1, vs. mediana 0, rango 25-75%: 0-0 en SLEout; p = 0,04). Conclusiones: Los pacientes SLEin fueron inicialmente más activos, requirieron mayores dosis de glucocorticoides e inmunosupresores, tuvieron una afectación renal más significativa y presentaron más daño y mayor mortalidad a corto plazo.

Vitamin D Levels and Steroid Usage are not Associated with Disease Activity in Systemic Lupus Erythematosus Patients

@#Introduction: Suboptimal vitamin D levels are commonly presented by systemic lupus erythemathosus (SLE) patients. This is likely due to protection measures from sunshine exposure adopted by SLE patients to reduce the likelihood of SLE flares onset. In this study, we investigated the vitamin D level among SLE patients and its association with SLE Disease Activity (SLEDAI) scores and among groups of steroid and non-steroid usage. Methods: We recruited 84 SLE patients who attended the Rheumatology Clinic of Hospital Universiti Sains Malaysia from June 2018 until October 2018. Their clinico-demographic data were retrieved and serum vitamin D immunoassay was conducted to measure the vitamin D levels of each patient Vitamin D levels were categorized as normal (≥75nmol/L), insufficient (50-74 nmol/L) or deficient (<50 nmol/L). Comparison between the clinico-demographic parameters with vitamin D levels were conducted using the Fisher’s exact test (for categorical variables) and unpaired t-test (for continuous variables). Results: The mean vitamin D level among the subjects was 40.79 ± 20.2 nmol/L. Fifty-eight (69%) patients were vitamin D deficient, while 20 (23.8%) patients were vitamin D insufficient, and only 6 (7.1%) patients had sufficient level of vitamin D. Vitamin D status was not significantly associated with SLEDAI score (p=0.185) as well as between steroids and non-steroids groups (p=0.255). Conclusion: Vitamin D deficiency occurred in majority of our SLE patients. SLE disease activities were not associated with the status of vitamin D or steroid usage.

Neutrophil-to-lymphocyte ratio in systemic lupus erythematosus is influenced by steroids and may not completely reflect the disease activity

Objectives: Neutrophil-to-lymphocyte ratio (NLR) has emerged as an important parameter in inflammatory network andcould pave the way for newer treatment strategies in systemic lupus erythematosus (SLE). The study evaluated NLR as apredictor of disease activity in SLE and attempted to relate the factors influencing the NLR.Methods: The study included 117 SLE patients fulfilling the systemic lupus international collaborating clinics/AmericanCollege of Rheumatology (SLICC/ACR) criteria (2010). The subjects were classified into mild, moderate, and severesystemic lupus erythematosus disease activity index 2000 (SLEDAI 2K) groups and compared. NLR values were classifiedas ≤2, >2–4 and >4 groups and its relationship with study variables was evaluated by Notched box-and-Whisker plots,Spearman correlation and Mountain plot. ROC and multiple linear regression were used to verify discriminatory abilityand factors influencing NLR respectively.Results: Approximately 24% (n=28) of patients each had mild and moderate SLEDAI disease activities, and 52.14% (n=61)had severe activity. Patients with severe disease activity were significantly younger (31.69±10.09 years) and were onmore immunosuppressants/DMARDs. The patients in the >4 NLR group had significantly elevated total leucocyte count(TLC) 5560 (3360-11480) cells/mm3 and CRP 4.46 (0.3-48.2) mg/L and more patients were on steroid therapy. The >2-4NLR group had moderate inverse correlation with SLEDAI. NLR, ESR, CRP, and C3 did not show agreement with SLEDAI.The NLR was associated with CRP and steroid usage and could not discriminate disease severity.Conclusion: The relationship of the NLR with SLEDAI was not consistent. NLR was associated with CRP and steroid use.NLR as a marker of inflammation or as a predictor of SLE disease activity needs further investigation.

A Clinical Study on NPSLE in a Tertiary Care Hospital in Northeast India

Introduction: Neuropsychiatric Systemic LupusErythematosus (NPSLE) is a disease with central andperipheral nervous system manifestations. The diagnosis ofNPSLE is often a diagnostic challenge though the criteria forNPSLE is well-established.Objective: To study the prevalence and pattern ofneuropsychiatric manifestations in SLE.Materials and Methods: This hospital based observationalstudy was carried out from Nov 2017 to June 2018. A total of58 cases of SLE were assessed clinically and investigatedaccordingly. Special emphasis was given to look forNeurological involvement. MMSE, HAM A and HAM D scaleswere used for assessment of cognitive dysfunction, anxiety anddepression respectively. The disease activity was measured bythe SLEDAI.Results: In a total of 58 patients with SLE evaluated, 92%were female. The most common age group was 21 to 30 years.Mean age was 25.6 years. Nervous system involvement wasfound in 37 (63.79%) patients. Headache was the mostfrequent manifestation, present in 32 (55.17%) patientsfollowed by Cognitive dysfunction in 8 patients (13.79%).Seizure disorder was present in 6 (10.34%), acute confusionalstate in 2 (3.44%), depression in 4 (6.89%), anxiety in 2(3.44%) and psychosis in 2 (26.31%) patients. Asepticmeningitis and peripheral neuropathy was found in 2 (3.44%)and 1 (1.72%) patients respectively. Many of the patients hadmore than one neurological involvement. SLEDAI score washigh in SLE patients with neurological manifestations.Conclusion: The frequency of Neuropsychiatric involvementwas found in the majority of the patients with SLE andheadache was the most common manifestation. Patients withNPSLE showed high disease activity.

Medición de la actividad lúpica y daño acumulado en pacientes con lupus eritematoso sistémico / Measurement of lupus activity and cumulative damage in patients with systemic lupus erythematosus

Introducción: medir la actividad de la enfermedad y el daño irreversible en pacientes con lupus eritematoso sistémico es de vital importancia para evaluar las medidas de desenlace del paciente y su pronóstico, las diferencias entre grupos de pacientes y las respuestas a nuevos tratamientos. Objetivo: determinar el grado de actividad lúpica y daño acumulado en un grupo de pacientes con lupus eritematoso sistémico. Método: se realizó un estudio observacional, descriptivo, de corte transversal, en el Hospital Universitario Mártires del 9 de Abril de Sagua la Grande, provincia Villa Clara, en el periodo comprendido de enero de 2013 a septiembre de 2018. La muestra, seleccionada de manera no probabilística por criterios, quedó conformada por 53 pacientes con diagnóstico de lupus eritematoso sistémico. Resultados: la media de puntajes de la escala MEX-SLEDAI fue de 5.8±3.7 puntos (rango 1-17 puntos). El 37.7 por ciento de los pacientes presentó actividad leve, sólo el 1.9 por ciento presentó actividad muy severa y el 17 por ciento se encontraba en remisión o sin actividad. Los dominios del índice SLICC/ACR más afectados fueron el musculoesquelético (17.0 por ciento), el cardiovascular (17.0 por ciento) y el cutáneo (15.1 por ciento). Conclusiones: los pacientes estudiados mostraron un predominio de actividad leve a moderada de la enfermedad según MEX-SLEDAI. La presencia de daño cardiovascular fue superior a la reportada por otros estudios. Se encontró correlación significativa entre la presencia de actividad lúpica y daño acumulado(AU)

Introduction: measuring the activity of the disease and the irreversible damage in patients with systemic lupus erythematosus is of vital importance to evaluate the outcome measures of the patient and their prognosis, the differences between groups of patients and the responses to new treatments. Objective: to determine the degree of lupus activity and accumulated damage in a group of patients with systemic lupus erythematosus. Method: an observational, descriptive, cross-sectional study was conducted at the Mártires del 9 de Abril University Hospital in Sagua la Grande, Villa Clara province, in the period from January 2013 to September 2018. The sample, selected in a non-probabilistic manner by criteria, was composed of 53 patients with a diagnosis of systemic lupus erythematosus. Results: the mean score of the MEX-SLEDAI scale was 5.8 ± 3.7 points (range 1-17 points). 37.7 percent of the patients presented mild activity, only 1.9 percent presented very severe activity and 17 percent were in remission or without activity. The most affected areas of the SLICC / ACR index were musculoskeletal (17.0 percent), cardiovascular (17.0 percent) and cutaneous (15.1 percent. Conclusions: the patients studied showed a predominance of mild to moderate disease activity according to MEX-SLEDAI. The presence of cardiovascular damage was higher than that reported by other studies. A significant correlation was found between the presence of lupus activity and accumulated damage(AU)

Correlation of Disease Activity with Primary Syndromes of Deficiency and Excess Types of Lupus Nephritis / 广州中医药大学学报

Objective To explore the correlation of disease activity with the primary syndromes of deficiency and excess types of lupus nephritis(LN). Methods A retrospective study was carried out in 134 LN patients,and the LN patients were differentiated into deficiency syndrome(deficiency group)and excess syndrome(excess group). The disease activity related indicators of systemic lupus erythematosus disease activity index(SLEDAI)scores, complements, inflammation indexes, and urine indicators were compared between the two groups. Results Of the 134 LN patients,33 cases were differentiated into excess syndrome,and 101 cases were differentiated into deficiency syndrome. According to the SLEDAI scores, 9.09％ of the patients in excess group were inactive, 4.95％ of the patients in deficiency group were inactive, and most of the patients in the two groups were at severe active stage. There was no significant difference of SLEDAI scores between the two groups(P>0.05) . The differences of complement C3 and C4 , inflammation indexes of C-reactive protein(CRP)and erythrocyte sedimentation rate(ESR),urine protein,and urine occult blood were also insignificant between the two groups (P>0.05). Conclusion There is no correlation between deficiency-excess syndrome differentiation and disease activity of LN. Most of the LN patients with deficiency syndrome are at active stage,but not at remission stage.

Self-Reported Physical Activity Is Associated with Lupus Nephritis in Systemic Lupus Erythematosus: Data from KORean Lupus Network (KORNET) Registry

PURPOSE: The aim of this study was to identify the associations among physical activity, disease activity, and organ damage in patients with systemic lupus erythematosus (SLE). MATERIALS AND METHODS: A total of 415 patients with SLE were consecutively enrolled from the KORean lupus Network (KORNET) registry. This registry assessed clinical features, disease activity [Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K)], and organ damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI)] upon enrollment in the study. Self-reported physical activity was measured by the International Physical Activity Questionnaire. Statistical analyses were conducted using the Mann-Whitney U test and multivariate logistic regression analysis. RESULTS: A significant difference in vigorous activity was noted between patients with lupus nephritis (LN) (n=93) and those without LN (n=322) (p=0.012), but not in moderate and walking activities. In contrast, no differences in physical activity, walking, moderate, and vigorous intensity, according to SLEDAI-2K and SDI were found. In addition to younger age (p=0.032), high physical component summary of SF-36 (p=0.004) and SLEDAI-2K (p=0.038), and less vigorous physical activity were associated with LN (p=0.024). However, cardiovascular disease was not associated with physical activity in SLE patients. CONCLUSION: This study showed that patients with LN had less vigorous physical activity than patients without LN. The results suggest that lupus nephritis might be associated with physical activity.

Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus / Vasculite digital inicial em uma grande coorte multicêntrica de pacientes com lúpus eritematoso sistêmico de início na infância

Abstract Objectives: To assess clinical digital vasculitis (DV) as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE) within a large population. Methods: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. Results: DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%). A poor outcome, with digital resorption, occurred in 5 (20%). Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p = 0.008), discoid rash (16% vs. 4%, p = 0.017), photosensitivity (76% vs. 45%, p = 0.002) and other cutaneous vasculitides (80% vs. 19%, p < 0.0001), whereas the frequency of overall constitutional features (32% vs. 61%, p = 0.003), fever (32% vs. 56%, p = 0.020) and hepatomegaly (4% vs. 23%, p = 0.026) were lower in these patients. Frequency of female gender, severe multi-organ involvement, autoantibodies profile and low complement were alike in both groups (p > 0.05). SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28) vs. 14 (0-58), p = 0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p = 0.014) was significantly lower in the DV group. Conclusion: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients.

Resumo Objetivos: Avaliar a vasculite digital (VD) clínica como uma manifestação inicial do lúpus eritematoso sistêmico de início na infância (LESi) em uma grande população. Métodos: Estudo transversal multicêntrico que incluiu 852 pacientes com LESi (critérios do ACR), acompanhados em dez centros de reumatologia pediátrica do Estado de São Paulo. Resultados: Observou-se VD em 25/852 (3%) pacientes com LESi. Diagnosticaram-se hemorragia periungueal em 12 (48%), infarto periungueal em sete (28%), úlcera de ponta de dígito em quatro (16%), nódulos dolorosos em um (4%) e gangrena em um (4%). Um desfecho ruim, com reabsorção digital, ocorreu em cinco (20%) pacientes. A comparação entre pacientes com e sem VD revelou maior frequência de erupção malar (80% vs. 53%, p = 0,008), erupção discoide (16% vs. 4%, p = 0,017), fotossensibilidade (76% vs. 45% p = 0,002) e outras vasculites cutâneas (80% vs. 19%, p < 0,0001), enquanto a frequência de características constitucionais totais (32% vs. 61%, p = 0,003), febre (32% vs. 56% p = 0,020) e hepatomegalia (4% vs. 23%, p = 0,026) foram menores nesses pacientes. A frequência do gênero feminino, o envolvimento grave de múltiplos órgãos, perfil de autoanticorpos e baixo complemento foram semelhantes nos dois grupos (p > 0,05). A mediana no Sledai-2 K, exclusive o descritor de VD, foi significativamente menor nos pacientes com VD em comparação com aqueles sem essa manifestação [10 (0 a 28) vs. 14 (0 a 58), p = 0,004]. Não foram observadas vasculite visceral nem morte nessa coorte de pacientes com LESi. A frequência de uso de ciclofosfamida (0% vs. 18%, p = 0,014) foi significativamente menor no grupo VD. Conclusão: Este grande estudo multicêntrico identificou a VD clínica como uma rara manifestação inicial do LESi ativo, associada a doença multissistêmica leve, apesar da ocorrência de reabsorção digital em alguns desses pacientes.

Disfunción cognitiva en lupus eritematoso sistémico y su asociación con actividad y daño / Cognitive dysfunction in systemic lupus erythematosus in relation to disease activity and damage

El lupus eritematoso sistémico (LES), es una enfermedad reumatológica, que puede presentar en su evolución alteraciones neurocognitivas, con importante compromiso fronto-subcortical. Sin embargo, existe escasa evidencia publicada sobre la relación entre la disfunción cognitiva y la actividad y daño de la enfermedad sistémica. El objetivo del trabajo fue analizar dicha asociación. Se realizó un estudio observacional de corte transversal, incluyendo 84 pacientes con LES. Se evaluó la actividad con el índice de SLEDAI y el daño acumulado con el índice de SLICC. Mediante pruebas neuropsicológicas se evaluó la presencia de síntomas cognitivos, función cognitiva global, memoria verbal y visual, viso-construcción, fluencia verbal semántica, velocidad de procesamiento y memoria de trabajo. Se consideró disfunción en un área cognitiva a un rendimiento de más de 1.5 desvíos estándares por debajo de los valores normales del test neuropsicológico. Se observó asociación estadísticamente significativa entre un mayor valor de SLEDAI y la alteración en la memoria de trabajo, y un mayor valor de SLICC y el compromiso de la viso-construcción y la fluencia verbal semántica. La asociación observada en los pacientes con LES entre el grado de actividad o daño de la enfermedad con algunos dominios cognitivos podría estar involucrando diferentes mecanismos fisiopatogénicos de la disfunción cerebral de cada área con distinto grado de afectación o vulnerabilidad.

Systemic lupus erythematosus (SLE) is a rheumatic disease, which during its evolution may present neurocognitive dysfunction with fronto-subcortical compromise. However, there is no enough published evidence regarding the relationship between cognitive dysfunction and SLE activity and SLE induced damage. The objective of the study was to analyze this association. We designed an observational cross-sectional study including 84 patients with SLE. We used the SLEDAI index to evaluate activity and the SLICC index to evaluate cumulative damage. We used neuropsychological tests to assess the presence of cognitive symptoms, global cognitive function, verbal and visual memory, visual-construction, semantic verbal fluency, processing speed and working memory. Scores more than 1.5 standard deviations below adjusted normal values were considered as cognitive dysfunction. We observed a statistically significant association between the higher value of SLEDAI and working memory impairment and a higher value of SLICC and viso-construction and semantic verbal fluency impairment. The association observed in SLE patients between disease activity or damage and some cognitive domains may be involving different pathophysiological brain mechanisms of different areas with different degrees of severity and vulnerability.

Cytokine profile in childhood-onset systemic lupus erythematosus: a cross-sectional and longitudinal study

Childhood-onset systemic lupus erythematosus (cSLE) exhibits an aggressive clinical phenotype and severe complications. This could be due to a pro-inflammatory cytokine milieu. Therefore, we determined plasma levels of Th1 (IL-2, IFN-γ, TNF), Th2 (IL-4), Th17 (IL-17A, IL-6), and Treg (IL-10) cytokines in a cohort of cSLE patients and healthy controls, and we evaluated the association between these cytokines and disease activity. We conducted a cross-sectional study with 51 cSLE patients from two pediatric rheumatology services. Ten cSLE patients participated in a longitudinal follow-up study. Blood samples were collected from the same patient during active and inactive disease. Disease activity was evaluated according to SLE Disease Activity Index 2000 (SLEDAI-2K). Cytokines levels were measured by cytometric bead array technique. cSLE patients had higher IL-6 (P<0.001) and IL-10 (P<0.001) levels than healthy controls. Patients with active disease had higher IL-6 and IL-10 levels than patients with inactive disease (P=0.001 and P=0.014, respectively) and the control group (both P<0.001). IL-6 (P=0.022), IL-10 (P=0.013), and IL-17A (P=0.041) levels were significantly higher during active than inactive disease. Linear regression analysis revealed IL-6 (P=0.002, 95%CI=0.006-0.025) and IL-10 (P=0.01 95%CI=0.021-0.150) as independent factors for increased SLEDAI-2K. IL-6, IL-10, and IL-17A are candidate biomarkers for disease activity in cSLE patients. This is the first longitudinal study to support their pivotal role in the pathogenesis of the disease.

Expression and clinical relevance of IRAK-M in monocytes in patients with systemic lupus erythematosus / 中国免疫学杂志

Objective:To study the expression and clinical relevance of IRAK-M in monocytes in patients with systemic lupus erythematosus. Methods: Real-time quantitative PCR was performed for IRAK-M mRNA measurement and enzyme-linked immunosorbent assay was used for anti-double-stranded DNA antibody ( dsDNA ) and anti-single-stranded DNA antibody ( ssDNA ) . Dynamic scattering turbidimetric immunoassay was applied for complement 3(C3),complement 4(C4) and C-reactive protein(CRP), while Westergren method for erythrocyte sedimentation rate (ESR). Correlation analysis of IRAK-M with SLEDAI,dsDNA,ssDNA,C3, C4,CRP and ESR was computed by Pearson or Spearman. Results:①The result showed that the mRNA expression of IRAK-M in SLE patients was significantly lower than healthy controls (P0. 05). Conclusion: This study indicated that IRAK-M play certain significant roles in the pathogenesis of SLE. We can monitor SLE disease activity and prognosis by quantitative detection of mRNA expression of IRAK-M. Meanwhile,it is very necessary to routinely test and regularly monitor the levels of dsDNA,ssDNA,C3,C4,CRP and ESR in SLE.

Expression and clinical significance of PD-L2 molecule on monocytes of peripher-al blood in systemic lupus erythematosus / 中国免疫学杂志

Objective:To analyze PD-L2 expression on monocytes of peripheral blood cells in systemic lupus erythematosus ( SLE) and it′s correlation with the degree of disease activity .Methods:Peripheral blood of 26 cases of SLE patiens and 38 cases of healthy controls were collected .Peripheral blood mononuclear cells ( PBMC) were isolated and realtime PCR was carried on to analyze the PD-L2 gene expression.At the same time flow cytometry was performed to analyze the CD 14 and PD-L2 expression.Results:PD-L2 was significantly up-regulated on monocytes in RA patients than in healthy controls and had correlation with the disease activity and the SLEAI score.Conclusion:These findings help to clarify the function of PD-L2,including its potential role as a biomarker for SLE .

Anticuerpos antinucleosoma frente a marcadores inmunológicos convencionales en el diagnóstico de la actividad del lupus eritematoso sistémico / Anti-nucleosome antibodies against conventional Immunological markers in the diagnosis of SLE activity

OBJETIVOS: examinar el perfil de anticuerpos en pacientes con lupus eritematoso sistémico y establecer la correlación entre los niveles de anticuerpos y la actividad de la enfermedad y de la nefritis lúpica. MÉTODOS: en 213 pacientes con lupus eritematoso sistémico, atendidos de forma consecutiva, se determinaron los anticuerpos anti-DNA de doble cadena (DNAdc), antinucleosoma (Nu), anti-Sm, anti-RNP, anti-Ro, anti-La y anti-Scl-70 por ensayo inmunoadsorbente ligado a enzima (ELISA), el C3 y el C4. La actividad de la enfermedad fue evaluada por el índice SLEDAI-2K, sin la contribución de anti-DNAdc, C3 ni C4. Los niveles de anticuerpos, el complemento y la actividad del lupus eritematoso sistémico fueron correlacionados. RESULTADOS: los niveles de todos los anticuerpos resultaron superiores significativamente en los pacientes en fase activa de la enfermedad con respecto a los que se hallaban en la fase inactiva. Los coeficientes de correlación de Spearman más altos con la puntuación de SLEDAI-2K correspondieron a los anti-Nu y anti-DNAdc (p= 0,856 y p= 0,616, respectivamente, p < 0,001 para ambos). Las áreas bajo de la curva (AUC) del análisis COR de la actividad del LES fueron en orden decreciente para los anti-Nu= 0,948, anti-DNAdc= 0,810, anti-RNP= 0,705, C4= 0,704, anti-Sm= 0,703, C3= 0,688, anti-Scl-70= 0,611, anti-La= 0,601 y anti-Ro= 0,593; y de la actividad renal para los anti-Nu= 0,845, anti-DNAdc= 0,755, C4= 0,694, C3= 0,670, anti-Sm= 0,641, anti-RNP= 0,630, anti-Scl-70= 0,611, anti-Ro= 0,593 y anti-La= 0,567. CONCLUSIÓN: los anticuerpos anti-Nu mostraron una capacidad discriminatoria excelente para la actividad del LES y de la actividad renal lúpica, superior a la de los anti-DNAdc y el resto de los marcadores.

OBJECTIVES: to examine the profile of antibodies in patients with systemic lupus erythematosus and establish the correlation between antibody levels and disease activity, and lupus nephritis. METHODS: anti-double stranded DNA (dsDNA), antinucleosoma (Nu), anti-Sm, anti-RNP, anti-Ro, anti-La, and anti-Scl-70 antibodies by enzyme-linked immunosorbent assay (ELISA) as well as C3 and C4 were determined in 213 patients with systemic lupus erythematosus who were treated consecutively. Disease activity was assessed by the SLEDAI 2K-index, without the contribution of anti-dsDNA, C3 or C4. The levels of antibodies, complement and activity of systemic lupus erythematosus were correlated. RESULTS: the levels of antibodies turned out to be remarkably higher in patients in the active phase of the disease compared to those who were in the inactive phase. The higher Spearman correlation coefficients with SLEDAI-2K score corresponded to the anti-Nu and anti-dsDNA (p = 0.856 and p = 0.616, respectively, p <0.001 for both). The areas under the ROC curve analysis of the activity of systemic lupus erythematosus were in descending order as follows: anti-Nu = 0.948; anti-dsDNA = 0.810; anti-RNP = 0.705; C4 = 0.704; anti-Sm = 0.703; C3 = 0.688; anti-Scl-70 = 0.611; = 0.601 anti-La. Anti-Ro = 0.593; and renal activity for anti-Nu = 0.845; anti-dsDNA = 0.755; C4= 0.694; C3 = 0.670; anti-Sm = 0.641; anti-RNP = 0.630; anti-Scl-70 = 0.611; anti-Ro = 0.593 and anti-La = 0.567. CONCLUSION: the anti-Un exhibit excellent activity discriminating capacity of SLE lupus and renal activity, higher than the anti-dsDNA and other markers.

Olfactory function and related factors in systemic lupus erythematosus / 中华风湿病学杂志

Objective To investigate the olfactory function in patients with systemic lupus erythematosus (SLE) and to explore factors that may influence it.Methods The Connecticut Chemosensory Clinical Research Center (CCCRC) test was carried out in SLE patients and healthy controls for olfactory function testing.ELISA method was used to detect anti-ribosomal P protein antibody in the serum.The statistical methods used in this study including t test,ANOVA,LSD-t test,Pearson correlation analysis,multiple linear regression analysis,andx2 test.Results ① The CCCRC scores of the active,inactive group and healthy controls were compared,the difference was statistical significant (F=26.52,P=0.01).CCCRC score in active SLE group (62±16) was lower than that of the inactive group (80±13) the and the normal control group (83±12) (P＜0.01),while there was no statistical significant difference between the inactive group and the normal controls (P=0.226).② CCCRC score was lower in 16 ARPA positive SLE patients (61±17) than 49 negative patients (74±16) (t=2.681,P=0.009).③ In addition,CCCRC score showed a negative correlation with ARPA serum concentration (r=-0.327,P=0.008).There was no significant correlation between CCCRC score and disease course (r=0.141,P＞0.05).④ Multiple linear regression analysis indicated that CCCRC score was associated with age and ARPA.Conclusion SLE patients have olfactory dysfunction and the dys-function is associated with age and ARPA.

Sintomas psiquiátricos em pacientes com lúpus eritematoso sistêmico: frequência e associação com atividade da doença com o uso do Questionário de Morbidade Psiquiátrica em Adultos / Psychiatric symptoms in patients with systemic lupus erythematosus: frequency and association with disease activity using the Adult Psychiatric Morbidity Questionnaire

INTRODUÇÃO: Enquanto os critérios de neurolúpus estão bem-estabelecidos, manifestações psiquiátricas globais são de frequência variável em pacientes com lúpus eritematoso sistêmico (LES); suas relações com atividade da doença e prognóstico são desconhecidas. OBJETIVO: Avaliar a frequência de sintomas psiquiátricos no LES utilizando o Questionário de Morbidade Psiquiátrica em Adultos (QMPA); correlacionar alterações no QMPA com atividade da doença e variáveis socioeconômicas. MATERIAIS E MÉTODOS: Este estudo transversal avaliou pacientes com LES ativo ou inativo quanto à prevalência de sintomas psiquiátricos utilizando, pela primeira vez, o QMPA. Oito ou mais respostas afirmativas entre 45 perguntas definiram um QMPA como anormal. Os pacientes foram classificados de acordo com os critérios do American College of Rheumatology de 1997, e o grau de atividade da doença foi mensurado pelo SLEDAI. RESULTADOS: Participaram do estudo 72 pacientes com LES, dos quais 68 eram do sexo feminino (94,4%). A média de idade foi de 46,1 anos (± 12 DP). A frequência de QMPA anormal foi de 89%. Entre os 64 pacientes lúpicos com QMPA alterado, 60 (93,7%) apresentavam distúrbios mentais comuns, a maioria ansiedade e somatização. Não houve correlação de sintomas psiquiátricos com atividade da doença (P = 0,46; rs = 0,09) ou com história de psicose e/ou convulsões atribuíveis ao LES (P = 1,00). Sintomas psiquiátricos também não se correlacionaram com idade de início da doença (rs = -0,16) ou duração da doença (rs = -0,11). Houve associação de QMPA anormal com baixo nível educacional (P=0,02), mas não com renda familiar destinada ao paciente (P = 0,24). CONCLUSÃO: A frequência de sintomas psiquiátricos medidos pelo QMPA foi alta em nossa população com LES. Um QMPA anormal esteve dissociado da atividade do LES, mas se associou com baixo nível educacional.

INTRODUCTION: While the neurolupus criteria are well-established, global psychiatric manifestations are of variable frequency in patients with systemic lupus erythematosus (SLE); their relation with disease activity is unknown. OBJECTIVE: To evaluate the frequency of psychiatric symptoms in SLE using the Adult Psychiatric Morbidity Questionnaire (APMQ); to correlate APMQ changes with disease activity and socio-economic variables. MATERIALS AND METHODS: This cross-sectional study evaluated patients with active or inactive SLE as to the prevalence of psychiatric symptoms utilizing, for the first time, the APMQ. Eight or more affirmative replies out of 45 questions defined the APMQ as abnormal. Patients were classified according to the American Collge of Rheumatology 1997 criteria, and disease activity was measured by the SLEDAI. RESULTS: Seventy-two SLE patients entered the study, being 68 females (94.4%). Mean age was 46.1 years (± 12 SD). The frequency of abnormal APMQ was of 89%. Out of the 64 SLE patients with altered APMQ, 60 (93.7%) had common mental disorders, mostly anxiety and somatization. There was no correlation of psychiatric symptoms with active disease (rs = 0.09; P = 0.46), or with history of psychosis and/or seizures attributable to SLE (P = 1.00). Psychiatric symptoms also did not correlate with age at disease onset (rs = -0.16) or disease duration (rs = -0.11). There was an association of abnormal APMQ with low education level (P = 0.02), but not with family income allotted to the patient (P = 0.24). CONCLUSION: The frequency of psychiatric symptoms measured by the APMQ was high in our SLE population. An abnormal APMQ was disconnected from SLE activity, but it did associate with low education level.

Clinical and serological manifestations associated with interferon-α levels in childhood-onset systemic lupus erythematosus

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 firstdegree 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 firstdegree 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.

The analysis of key indicators what may affect the acticity index in systemic lupus erythematosus / 中华风湿病学杂志

Objective To investigate the distribution of characteristics,clinical manifestations,laboratory parameters and activity index of patients with systemic lupus erythematosus (SLE) and to determine their role in helping to make correct clinical diagnosis and disease the activity evaluation of SLE.Methods Collect the data of 1037 SLE patients of Ningxia Medical University Affiliated Hospital from January 2006 to June 2010.Data were analyzed with t test and Chi-square test.Results Over the past three years,there were more and more patients were admitted year by year.Among the 1037 cases of SLE patients,most of them 20-40 year-old woman,accounting for 67.5％ of the whole patient population,with a male to female ratio was 1:8.26.Joint pain was the most common initial symptom,accounting for 54.3％,followed by skin rash,accounting for 48.2％.Decreased complement C3 level and platelets counts, proteinuria,and positive anti-dsDNA antibody could be used as indicators for early diagnosis of SLE.SLEDAI activity score higher than 9 were presented in 26.0％ of patients.Factors that could impact the final score of SLEADI were fever,arthritis,skin rash,proteinuria,low complement levels,high titers of anti-dsDNA antibody,pleurisy,alopecia,mucosal ulcers,pericarditis,mental illness and decreased platelets count.Patients with active disease had a higher accidence of fever,arthritis,skin rash,lung damage,alopecia,mucosal ulcers,heart damage,mental illness and renal damaged,low complement levels,high level of anti-dsDNA antibody titers and elevated erythrocyte sedimentation rate.Conclusion SLE is a multi-system disease with multiple organ involvement,with characteristic clinical symptoms and immunological abnormalities,thus early diagnosis is very important.Understanding the characteristics of the diseases,correct judgement of the disease activity,reasonable and effective treatment all can delay the development of organ damage and improve the prognosis.

Correlations study between cutaneous disease severity and systemic disease severity in SLE (Systematic Lupus Erythematosus) patients

Introduction: Skin is the second most commonly affected organ in SLE. Lupus-specific cutaneous LE(CLE) is classified according to Gilliam Classification into acute CLE (ACLE), subacute CLE(SCLE), chronic CLE(CCLE). CLASI (CLE Disease Activity and Severity Index) is an outcome measures to asses cutaneous activity Objectives: To study the correlation between cutaneous disease severity and severity of systemic disease using SLEDAI (SLE Disease Severity Index) and SLICC / ACR (Systemic Lupus International Collaborating Clinics) outcome measures. Methods: Study design: Cross-sectional A total of 71 patients were recruited from Dermatology and Rheumatology Clinic from Kuala Lumpur Hospital, Selayang Hospital and Pusat Perubatan UKM. Study period was from December 2009 to August 2010. Study data were obtained from clinical history, examination, investigations and medical record review. Results: The mean CLASI activity/damage scores in patients with ACLE, SCLE and CCLE were 11.8 / 8.1, 22.6 / 17.2 and 21.1 / 22.1 respectively. The mean SLEDAI/SLICC scores in similar group of patients were 12.3 / 2.1, 6.8 / 1.6, 13.2 / 1.7 respectively. The cutaneous disease activity in patients with SCLE was found to be inversely correlated to systemic disease activity. The cutaneous damage in patients with CCLE was positively correlated with systemic disease damage Conclusions: Patients with SCLE, despite having high cutaneous disease activity, had mild systemic disease. Patients with CCLE whom had high cutaneous damage scores were more likely to have higher systemic damage, hence more thorough investigations to seek other organs damage, should be offered.

Estudo longitudinal do anticorpo antilipoproteína lipase e sua relação com atividade de doença nos indivíduos com lúpus eritematoso sistêmico sem anticorpos anti-dsDNA / Longitudinal fluctuation of anti-lipoprotein lipase antibody is related with disease activity in systemic lupus erythematosus patients without anti-dsDNA antibodies

INTRODUÇÃO: O Lúpus Eritematoso Sistêmico (LES) se caracteriza por períodos de exacerbação e remissão clínica que frequentemente são acompanhados por alterações nos níveis séricos de anticorpos específicos, como o anti-dsDNA, que está presente em 40 por cento dos casos, associado principalmente à atividade renal. Recentemente houve a descrição de duas subpopulações de anticorpos antilipoproteína lipase (anti-LPL) no LES: uma com e a outra sem atividade anti-dsDNA. A possível relação desse último grupo de anticorpos com a atividade inflamatória de doença ainda não foi analisada no LES. OBJETIVOS: Avaliar longitudinalmente a associação dos níveis séricos dos anticorpos anti-LPL com atividade do LES em pacientes com anti-dsDNA persistentemente negativo. PACIENTES E MÉTODOS: Cinco pacientes com LES com anti-dsDNA persistentemente negativo mensurado por ELISA e por imunofluorescência indireta em crithidia luciliae e altos títulos de anti-LPL por ELISA (> 5 desvios-padrão (DP) da média de 20 controles normais) foram selecionados e acompanhados longitudinalmente durante um período mínimo de dois anos. RESULTADOS: Caso 1: Homem, 24 anos com LES desde 2001 apresentou hemorragia alveolar, proteinúria, hipertensão arterial sistêmica, eritema malar, aftas, artrite, FAN+, com SLEDAI (systemic lupus erythematosus disease activity index) = 16 e anti-LPL = 144UA. Tratado com pulso de metilprednisolona e prednisona com melhora clínica e SLEDAI = 0 e redução do anti-LPL (109UA). Nova atividade com acometimento renal em abril de 2002, SLEDAI = 10 e aumento de anti-LPL (150UA). Iniciada pulsoterapia de ciclofosfamida e metilprednisolona com boa resposta, SLEDAI = 0 e diminuição de anti-LPL (77UA) até a sua total negativação acompanhando a remissão do quadro no ano de 2003. Caso 2: Mulher, 32 anos, com LES desde 1997. Em setembro de 2001 iniciou vasculite cutânea, febre e rash, SLEDAI = 10, anti-LPL = 80UA. Em janeiro de 2002, teve atividade renal e HAS...

INTRODUCTION: Systemic lupus erythematosus (SLE) is characterized by periods of clinical flares and remission that are followed by alterations of sera specific autoantibodies such as anti-dsDNA, present in 40 percent of the cases and strongly associated with renal involvement. Recently, there was a description of two subpopulations of anti-lipoprotein lipase antibodies (anti-LPL) in SLE: with and without anti-dsDNA activity. A possible relationship between these antibodies with inflammatory activity of SLE was not analyzed. OBJECTIVES: To evaluate longitudinally the association between anti-LPL with lupus activity in patients persistently negatives for anti-dsDNA antibodies. PATIENTS AND METHODS: Five SLE patients with persistently negative anti-dsDNA measured by ELISA and indirect immunofluorescence using crithidia luciliae and high titers of anti-LPL by ELISA (> 5 SD) were selected and followed for at least 2 years. RESULTS: Case 1: A 24-year-old male with SLE since 2001, presented with alveolar hemorrhage, proteinuria, systemic hypertension, malar rash, oral ulcers, polyarthritis, positive ANA, SLEDAI=16 and anti-LPL=144U. He was treated with intravenous (IV) methylprednisolone followed by prednisone and had an excellent response. SLEDAI=0, anti-LPL decreased to 109U. New renal flare in April 2002, SLEDAI=10 and a new increment of anti-LPL (150U). IV Cyclophosphamide and methylprednisolone were started and he achieved remission, SLEDAI=0 and a decrease of anti-LPL (77U) until become negative in 2003. Case 2: A 32-year-old female had SLE since 1997. In September 2001 began cutaneous vasculitis, fever and rash, SLEDAI=10, anti-LPL=80U. In January 2002, she had renal involvement and systemic hypertension, SLEDAI=8 and anti-LPL= 25U. She received corticosteroid and cyclophosphamide and improved. In 2003, she was asymptomatic, SLEDAI=2 and anti-LPL=12U. Case 3: A 39-year-old male has SLE since 1997. He was stable, under chloroquine use...

Prevalência de microrganismos potencialmente superinfectantes na cavidade bucal de pacientes com Lúpus Eritematoso Sistêmico / Prevalence of potentially superinfectant microorganisms from the oral cavity of systemic erithematous lupus

Considerando-se que pacientes com Lúpus Eritematoso Sistêmico são submetidos a tratamento com corticosteróides e imunossupressores, podemos concluir que esta condição pode interferir na presença de microrganismos potencialmente oportunistas na cavidade bucal. Deste modo, o objetivo deste estudo foi avaliar a presença de leveduras do gênero Candida, estafilococos, enterobactérias e Pseudomonas spp. na cavidade bucal de pacientes com LES comparando os resultados com indivíduos controle. Desta forma, foram selecionados 40 pacientes com idades entre 19 e 53 anos com LES e que estavam em tratamento por no mínimo 60 dias. Para o grupo controle foram selecionados 40 indivíduos sistemicamente saudáveis com perfil semelhante (quanto à idade, gênero e condições bucais) aos pacientes do grupo em estudo. Não foram incluídos pacientes diabéticos, portadores de próteses bucais totais e outras doenças sistêmicas e que estavam sob terapia com medicamentos que pudessem interferir com as condições bucais. Foram realizados anamnese, exame clínico e coleta de enxágüe bucal de cada paciente. A amostra de enxágüe bucal foi semeada em meios de cultura específicos para cada microrganismo e após período de incubação foram realizadas contagens de unidades formadoras de colônia (UFC), obtendo-se o número de UFC/mL. A partir dos isolados obtidos, foram realizadas provas de identificação a fim de caracterizar as espécies dos gêneros em estudo. As contagens de microrganismos foram comparadas entre os grupos LES e controle por ANOVA, Mann Whitney (5%). Contagens de microrganismos em indivíduos sob tratamento com fármacos imunossupressores ou não e atividade positiva ou negativa da doença (SLEDAI) também foram comparadas. Não foram observadas diferenças significativas nas contagens de microrganismos entre os grupos em estudo (leveduras, p=0,55; estafilococos, p=0,24; enterobactérias/Pseudomonas spp., p=0,26)...

Considering that patients with systemic erithematous lupus are treated with corticoids and immunossuppresive drugs, this condition may interfere in the presence of potentially opportunistic microorganisms in the oral cavity. The aim of this study was to evaluate the presence of Candida spp. staphylococci, enterobacteria and Pseudomonas spp. in the oral cavity of patients with systemic erithematous lupus (LES) comparing the results with control individuals. Forty patients aged 19-53 years with LES and under therapy for at least 60 days were selected. For the control group, 40 healthy individuals paired to the test group in relation to age, gender and oral conditions were selected. Diabetic and other systemic diseases patients, denture users and individuals under therapy with drugs that affect the oral conditions were not included. Clinical examination, anamnesis and oral rinses sampling were performed. Oral rinse samples were plated on specific culture media and after the period of incubation the number of colony forming units were counted, and the value of cfu/ml was obtained. The isolates were identified in order to obtain the species. The counts of microorganisms were compared between LES and control groups by ANOVA, Mann Whitney (5%). Also, counts of microorganisms in patients under treatment with immunossupressive drugs of not and positive or negative activity of the disease (SLEDAI) were compared. No significant differences in the counts of microorganisms between the studied groups were observed (yeasts, p= 0.55; staphylococci, p=0.24; enterobacteria/Pseudomonas spp., p=0.26). No differences in the counts of microorganisms were observed related to the clinical parameters tested. Higher prevalence of Candida albicans and Staphylococcus epidermidis was observed in LES and control group. Klebsiella oxytoca was the most frequently observed in the LES group and Enterobacter cloacae in thecontrol group.