ABSTRACT
Objective@#To describe the clinical profile of Filipino pediatric SLE patients as determined using the 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria. @*Methods@#We checked which among the SLICC criteria were fulfilled by Filipino pediatric SLE patients when we examined them and their case records, as part of a nationwide genetic study on SLE conducted from October 2015 to March 2017. @*Results@#Ninety-seven (out of 321) who were diagnosed to have SLE before 19 years of age were evaluated.The mean age of the population at the time of evaluation was 19.8 ± 6.9 years. Females comprised 94% of our population. Mean age of onset was 14.4 ± 2.7 years, while the mean age of diagnosis was at 14.5 ± 2.6 years. Acute cutaneous rash was found in 87%; oral ulcers 65%; renal disorder 63%; non-scarring alopecia 61%; arthritis 58%; chronic cutaneous rash 36%; leukopenia 35%; hemolytic anemia 34%; serositis 25%; thrombocytopenia 23%, and neurologic disorder 8%. Anti-nuclear antibody was present in 85%; low complement 32%; anti-dsDNA 28%; direct Coombs’ 16%; antiphospholipid antibody 3%; and anti-Smith antibody 1%. Kidney biopsy was performed in only 14% (14/97) of patients, of whom 27% had class III histopathologic characteristic.@*Conclusions@#Filipino pediatric SLE patients typically present with mucocutaneous, renal, and musculoskeletal involvement. Cardiopulmonary and neurologic manifestations are found to be less common among them. Finally, renal biopsy is not commonly performed among these patients.
Subject(s)
Child , Philippines , Lupus Erythematosus, SystemicABSTRACT
RESUMEN El daño irreversible de órgano es predictor de morbilidad, mortalidad, mayor acúmulo de daño y mala calidad de vida en los pacientes con lupus eritematoso sistémico. Objetivos: Caracterizar el daño y los factores que mejor lo explican, en una población de pacientes colombianos con lupus eritematoso sistémico. Métodos: Estudio retrospectivo de seguimiento a una cohorte. El daño se midió con el SLICC/ACR (índice de Systemic Lupus International Collaborating Clinics y del American College of Rheumatology) y la actividad de la enfermedad por SELENA SLEDAI. La caracterización del daño se hizo mediante estadística descriptiva, los factores asociados con el desenlace se evaluaron con Chi2 de Pearson o Fisher, t de Student o U de Mann-Whitney; la proporción de pacientes que acumularon daño se evaluó con el test de Friedman y el puntaje acumulado con el test de Wilcoxon. La determinación de los factores asociados independientemente con el desenlace se hizo con una regresión logística. Resultados: Se incluyeron 161 pacientes con diagnóstico de novo y seguimiento mínimo de un año; el 28,9% sufrió daño. Los dominios más representados fueron el neuropsiquiátrico, renal y vascular. Los anticuerpos antifosfolípido, las dosis promedio de prednisolona mayores a 12,5 mg/día y presentar 2 o más recaídas se asociaron independientemente al daño orgánico. Conclusiones: Los anticuerpos antifosfolípido, la dosis de esteroides y la frecuencia de recaídas se asocian al daño orgánico en una población colombiana de pacientes con lupus eritematoso sistémico.
ABSTRACT Irreversible organ damage is a predictive factor of morbidity, mortality, increased accumulation of damage, and poor quality of life in patients with systemic lupus erythematosus. Objectives: To describe the damage, and the factors that best explain it, in a population of Colombian patients. Methods: A retrospective follow-up study of a patient cohort. The damage was measured using the Systemic Lupus International Collaborating Clinics (SLICC) and the American College of Rheumatology (ACR) index, and disease activity by SELENA SLEDAI. Descriptive statistics were used to describe the damage. The factors associated with the outcome were evaluated with Pearson's or Fisher's Chi2, Student's t or Mann-Whitney's U. The proportion of patients that accumulated damage was evaluated with the Friedman test, and the cumulative score with the Wilcoxon test. The determination of the factors independently associated with the outcome was performed using logistic regression. Results: A total of 161 patients with recent diagnosis, and followed for one year or more, were included, 28.9% of whom had suffered damage. The most represented domains were neuropsychiatric, renal and vascular. Anti-phospholipid antibodies, mean doses of prednisolone greater than 12.5 mg/day, and suffering 2 or more relapses were independently associated with organ damage. Conclusions: Anti-phospholipid antibodies, steroid doses and frequency of relapses are associated with organ damage in a Colombian population of patients with systemic lupus erythematosus.
Subject(s)
Humans , Adolescent , Adult , Middle Aged , Aged , Aged, 80 and over , Lupus Erythematosus, Systemic , Quality of Life , Prednisolone , Antibodies, Antiphospholipid , Lupus Vasculitis, Central Nervous System , AntibodiesABSTRACT
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.
Subject(s)
Humans , Cardiovascular Diseases , Logistic Models , Lupus Erythematosus, Systemic , Lupus Nephritis , Motor Activity , Rheumatology , WalkingABSTRACT
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.
Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Aged , Young Adult , Cognitive Dysfunction/diagnosis , Cognitive Dysfunction/etiology , Lupus Erythematosus, Systemic/complications , Severity of Illness Index , Prevalence , Cross-Sectional Studies , Neuropsychological TestsABSTRACT
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.