ABSTRACT
El síndrome de activación macrofágica (SAM) presenta criterios clínicos y de laboratorio establecidos. Presentamos el caso de un adolescente varón con debut de Lupus eritematoso generalizado pediátrico grave, donde su manifestación principal fue un SAM y el receptor de interleucina 2 soluble en suero (IL-2rs) o CD25 soluble (CD25s) aumentado resultó clave en la confirmación diagnóstica, en el tratamiento y pronóstico de su enfermedad. Sin embargo, este receptor de citocinas no se mide habitualmente en la práctica clínica.
Macrophage activation syndrome (MAS) presents established clinical and laboratory criteria. We present the case of a male adolescent with the onset of severe pediatric systemic Lupus erythematosus, manifested mainly by MAS and how a laboratory marker, serum soluble interleukin-2 receptor (IL-2rs) or altered soluble CD25(CD25s), played a key role in treatment and prognosis of the disease. However, this cytokine receptor is rarely measured in clinical practice.
Subject(s)
Humans , Male , Child , Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/therapy , Macrophage Activation Syndrome/diagnosis , Macrophage Activation Syndrome/therapy , Thorax/diagnostic imaging , Radiography, Thoracic/methods , Receptors, Interleukin-2 , Macrophage Activation Syndrome/pathology , Lupus Erythematosus, SystemicABSTRACT
Abstract Objective To evaluate alcohol, smoking and/or illicit drug use, and history of bullying in adolescent childhood-onset systemic lupus erythematosus and healthy controls. Methods 174 adolescents with pediatric rheumatic diseases were selected. All of the 34 childhood-onset systemic lupus erythematosus patients and 35 healthy controls participated in this study. A cross-sectional study included demographic/anthropometric data and puberty markers assessments; structured questionnaire and CRAFFT screening interview. Results McNemar tests indicated an excellent test–retest reliability of the structured questionnaire (p = 1.0). The median current age was similar between childhood-onset systemic lupus erythematosus patients and controls [15 (12–18) vs. 15 (12–18) years, p = 0.563]. The median of menarche age was significantly higher in childhood-onset systemic lupus erythematosus patients compared to controls [12 (10–15) vs. 11.5 (9–15) years, p = 0.041], particularly in those that lupus had occurred before first menstruation [13 (12–15) vs. 11.5(9–15) years, p = 0.007]. The other puberty marker and sexual function parameters were similar in both groups (p > 0.05). Alcohol use was similar in both childhood-onset systemic lupus erythematosus patients and controls (38% vs. 46%, p = 0.628). A trend of lower frequency of CRAFFT score ≥2 (high risk for substance abuse/dependence) was evidenced in childhood-onset systemic lupus erythematosus patients compared to controls (0% vs. 15%, p = 0.053). Bullying was reported similarly for the two groups (43% vs. 44%, p = 0.950). Further analysis in lupus patients regarding alcohol/smoking/illicit drug use showed no differences in demographic data, puberty markers, history of bullying, sexual function, contraceptive use, disease activity/damage scores, clinical/laboratorial features and treatments (p > 0.05). Conclusion This study showed high frequencies of early alcohol use in lupus adolescents and healthy controls, despite of a possible low risk for substance abuse/dependence in childhood-onset systemic lupus erythematosus patients.
Resumo Objetivo Avaliar o uso de álcool, tabaco e/ou drogas ilícitas e a história de bullying entre adolescentes com lúpus eritematoso sistêmico pediátrico (LES-i) e controles saudáveis. Métodos Selecionaram-se 174 adolescentes com doenças reumatológicas pediátricas. Todos os 34 pacientes com LES-i e 35 controles saudáveis participaram deste estudo. Um estudo transversal incluiu avaliações de dados demográficos/antropométricos e marcadores da puberdade, um questionário estruturado e a entrevista de triagem Crafft. Resultados Testes de McNemar indicaram uma excelente confiabilidade teste-reteste do questionário estruturado (p = 1,0). A idade média atual foi semelhante entre pacientes com LES-i e controles [15 (12 a 18) vs. 15 (12 a 18) anos, p = 0,563]. A mediana da idade na menarca foi significativamente maior em pacientes com LES-i em comparação com os controles [12 (10 a 15) vs. 11,5 (9 a 15) anos, p = 0,041], particularmente naquelas em quem o lúpus ocorreu antes da primeira menstruação [13 (12 a 15) vs. 11,5 (9 a 15) anos, p = 0,007]. Os outros marcadores da puberdade e parâmetros de função sexual foram similares nos dois grupos (p > 0,05). O uso de álcool foi semelhante entre pacientes com LES-i e controles (38% vs. 46%, p = 0,628). Evidenciou-se uma tendência de menor frequência de pontuação ≥ 2 no Crafft (alto risco para uso abusivo/dependência de substâncias) em pacientes com LES-i em comparação com os controles (0% vs. 15%, p = 0,053). O bullying foi relatado em frequência semelhante nos dois grupos (43% vs. 44%, p = 0,950). Uma análise mais aprofundada em relação ao uso de álcool/tabaco/drogas ilícitas em pacientes com lúpus não mostrou diferenças nos dados demográficos, marcadores da puberdade, história de bullying, função sexual, uso de anticoncepcionais, escores de atividade/danos da doença, características clínicas/laboratoriais e tratamentos (p > 0,05). Conclusão Este estudo mostrou uma alta frequência de uso precoce de álcool em adolescentes com lúpus e controles saudáveis, apesar de um possível baixo risco para uso abusivo/dependência de substâncias em pacientes com LES-i.
Subject(s)
Humans , Male , Female , Child , Adolescent , Smoking/epidemiology , Substance-Related Disorders/epidemiology , Alcoholism/epidemiology , Lupus Erythematosus, Systemic , Sexual Maturation , Illicit Drugs , Case-Control Studies , Cross-Sectional Studies , Reproducibility of Results , BullyingABSTRACT
Foreword: Systemic Lupus Erythematosus (SLE) is an inflammatory, autoimmune and multisystemic disease featured by the presence of multiple antibodies, and which appears in both genders and at any age, including children, albeit rare under 5 years of age. Objective: To assess its clinical and in-lab manifestations, and the evolution of pediatrics patients and of those who transition into adulthood. Results: Seventeen (17) patients with infantile inception were assessed; they met the SLICC/ACR diagnose criteria over a time span running between years 2001 and 2013. They recorded an 88 percent of musculoskeletal compromise, 88 percent of renal compromise, 47 percent of alopecia, 47 percent of photosensitivity, and 42 percent of neurological compromise. All (100 percent) of them tested positive for antinuclear antibodies (ANA), 88 percent for anti-DNA, and 53 percent were Sm-positive. Anaemia was found in 42 percent of them, Leukopenia in 12 percent, and Lymphopenia in 35 percent. As regards the treatment, all of them (100 percent) received hydroxychloroquine, 94 percent were administered prednisone, 82 percent mycophenolate, and 71 percent cyclophosphamide. Fifteen of them (88 percent) have transitioned to the adultspolyclinic, and from them 6 (35 percent) have evidenced SLE reactivations associated to the lack of adherence to treatment. In this series the survival rate is of 100 percent of patients during the follow-up period. Conclusion: Infantile-inception Lupus, although less frequent, is more severe than that with an inception during adulthood, with a high prevalence of nephropathy, above half of the Classes III or IV. Early treatment with corticoids, immunosuppressants and hydroxychloroquine enables a favorable evolution and an appropriate transition into adulthood, with a survival rate of 100 percent. It is necessary to strengthen a good doctor-patient relationship during this transition, including a proper communication between the pediatric...
Introducción: El Lupus Eritematoso Sistémico (LES) es una enfermedad inflamatoria, autoinmune y multisistémica, caracterizada por la presencia de múltiples autoanticuerpos y que se presenta en ambos sexos y a cualquier edad, incluido en niños, siendo raro bajo los 5 años de edad. Objetivo: Evaluar sus manifestaciones clínicas, de laboratorio y evolución en pacientes pediátricos y en los que pasan a adultos. Resultados: Se analizó a 17 pacientes de inicio infantil que cumplieron los criterios diagnósticos SLICC y ACR en un periodo comprendido entre los años 2001 y 2013. Presentaron compromiso musculoesquelético 88 por ciento, renal 88 por ciento, alopecia 47 por ciento, fotosensibilidad 47 por ciento y neurológico 42 por ciento. Un 100 por ciento tuvieron anticuerpos antinucleares (AAN) positivos, 88 por ciento anti-DNA y 53 por ciento Sm positivos. Se encontró anemia en 42 por ciento, leucopenia en 12 por ciento y linfopenia en 35 por ciento. En relación al tratamiento, el 100 por ciento recibió hidroxicloroquina, prednisona 94 por ciento, micofenolato 82 por ciento y ciclofosfamida 71 por ciento. Quince (88 por ciento) han hecho la transición al Policlínico de Adultos, de los cuales seis (35 por ciento) han presentado reactivaciones del LES asociadas a falta de adherencia al tratamiento. En esta serie la sobrevida es del 100 por ciento de los pacientes durante el periodo de seguimiento. Conclusión: El Lupus de inicio infantil, si bien menos frecuente, es más severo que el de inicio adulto, con alta prevalencia de nefropatía, sobre la mitad de clase III o IV. El tratamiento precoz con corticoides, inmunosupresores e hidroxicloroquina permite una evolución favorable y un adecuado paso a la adultez, con 100 por ciento de sobrevida. Es necesario reforzar una buena relación médico-paciente en esta transición, incluida una comunicación adecuada entre el reumatólogo infantil y de adulto, que asegure mantener una adherencia y control óptimo de la enfermedad.
Subject(s)
Humans , Male , Adolescent , Female , Child , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/drug therapy , Retrospective StudiesABSTRACT
El lupus eritematoso sistémico pediátrico (LESp) es una enfermedad autoinmunitaria crónica con manifestaciones clínicas complejas y ciertos aspectos especiales que se deben considerar en la población pediátrica. La supervivencia ha mejorado notablemente en la última década gracias a un diagnóstico precoz e instauración de tratamientos tempranos y más agresivos. Sin embargo, su pronóstico continua siendo grave. Presentamos una resumen de esta patología y el caso de una paciente de 11 años que fue diagnosticada en el Hospital del Niñ@ "Manuel Ascencio Villarroel"con LESp Clase IV.
Pediatric systemic lupus (pSLE) is a chronic multisystemic autoinmune disease with complex clinical manifestations and special issues, which need to be considered when dealing with SLE in children. In the last decade, survival has improved remarkably as a result of earlier diagnosis and better aproaches therapy. However, it remains a potencially serious condition. We present a summary of this disease and 11 years oíd patient's case diagnosed at "Manuel Ascencio Villarroel" Child's Hospital.
