Lupus juvenil
Les debo confesar que el lupus eritematoso sistemico juvenil es el culpable de que haya hecho la sub especialidad en reumatología pediátrica, durante mi formación como pediatra, me enamore de la complejidad y la amplia variedad de presentación en los niños, mi asombro fue mayor al darme cuenta que en la laguna no había reumatologos pediatras, entonces me fui a la ciudad de México al mayor centro de preparación a nivel nacional , CENTRO MEDICO NACIONAL LA RAZA, y fue así que me convertí en la primera reumatologa pediatra en la región.
He diagnosticado y tratado varios casos de lupus en diferentes hospitales privados, e incluso a nivel institucional , que aunque trabajo como pediatra, eso no me impide echar mano de mis conocimientos como sub especialista en varios casos, el resultado, niños diagnosticados de manera temprana, tienen una mayor calidad de vida.
¿En qué consiste el lupus juvenil?
El lupus juvenil eritematoso sistémico (LES) es una enfermedad autoinmune crónica que puede afectar varios órganos del cuerpo, especialmente la piel, las articulaciones, la sangre, los riñones y el sistema nervioso central. El término «crónica» significa que puede durar mucho tiempo. «Autoinmune » significa que se trata de un trastorno del sistema inmunitario, que en lugar de proteger al cuerpo de las bacterias y los virus, ataca a los propios tejidos del paciente.
¿Cuáles son las causas de la enfermedad?
El LES no es contagioso. Es una enfermedad autoinmune, en la que el sistema inmune pierde su capacidad de distinguir entre las sustancias extrañas de las de los proprios tejidos o células. El sistema inmunitario comete un error y produce, entre otras sustancias, autoanticuerpos que identifican las células normales de la propia persona como si fuesen extrañas y las atacan. El resultado es una reacción autoinmunitaria, que da lugar a la inflamación de órganos específicos (articulaciones, riñones, piel, etc.).
Lupus juvenil puede producirse en familias. Los niños heredan algunos factores genéticos, aunque todavía desconocidos, de sus progenitores que pueden predisponerles a desarrollar LES.
Leer: Enfermedad reumática y calidad de vida
¿Cuáles son los síntomas?
Los primeros en aparecer son datos comunes y que se dejan pasar desapercibidos, pensado en gripe o cualquier otro proceso infeccioso pues tienen fiebre, cansancio, fatiga, pérdida de peso.
La afectación de la piel y de las mucosas es muy frecuente y puede incluir varias erupciones, fotosensibilidad (en la que la exposición a la luz del sol desencadena una erupción cutánea) o úlceras dentro de la nariz o de la boca. La erupción cutánea de tipo «mariposa» por la nariz y las mejillas se produce entre un tercio y la mitad de los niños afectados. En ocasiones, también puede percibirse una mayor pérdida de pelo (alopecia). Las manos se pueden volver rojas, blancas y azules cuando se exponen al frío (fenómeno de Raynaud). Los síntomas también pueden incluir inflamación y rigidez de las articulaciones, dolor muscular, anemia, fácil aparición de hematomas, dolores de cabeza, convulsiones y dolor en el pecho. La afectación de los riñones está presente en la mayoría de los niños con LES y es un determinante principal del desenlace a largo plazo de la enfermedad.
Hay que tomar en cuenta que el lupus en los niños se presenta de una manera mas agresiva y aparatosa que como se presenta en los adultos, de ahí que los reumatólogos pediátricos manejemos tratamientos mas “Fuertes y agresivos”.
El diagnóstico del LES se basa en una combinación de síntomas (como dolor), signos (como fiebre) y exámenes (de orina y de sangre); después de haber descartado otras enfermedades. No todos los síntomas y signos se presentan al mismo tiempo; esto hace que el diagnóstico de LES sea difícil de hacer con rapidez, ya que el pediatra o medico general no suele pensar en lupus en los niños.
¿Cuál es la importancia de los análisis?
Los exámenes de laboratorio pueden ayudar a diagnosticar el LES y a decidir qué órganos internos están afectados, en caso de haberlos. Los exámenes regulares de sangre y orina son importantes para supervisar la actividad y la intensidad de la enfermedad y para determinar lo bien que se toleran los medicamentos. Hay varios exámenes que pueden ayudar a diagnosticar LES y a decidir qué medicamentos recetar, así como a evaluar si los medicamentos recetados están funcionando bien para controlar la enfermedad, por lo que si!!! Son necesarias las visitas a laboratorio hasta mantener quieta la enfermedad donde los exámenes se piden mas espaciados.
¿Puede tratarse o curarse? En la actualidad, no hay un medicamento específico para curar el LES. El tratamiento de lupus juvenil ayudará a controlar los dignos y síntomas de esta enfermedad y ayudará a evitar sus complicaciones, incluido el daño permanente a los órganos y tejidos.
Cuando se diagnostica LES por primera vez, suele ser muy activo. En esta etapa, puede requerir grandes dosis de medicamentos para controlar la enfermedad y evitar el daño a los órganos. En muchos niños, el tratamiento mantiene bajo control los brotes de LES y la enfermedad puede entrar en remisión (inactividad), llegado ese momento se necesita poco o ningún tratamiento.
¿Existe algún tratamiento no convencional o complementario? Existen muchos tratamientos complementarios y alternativos disponibles y esto puede confundir a los pacientes y sus familiares. Piense con atención los riesgos y beneficios de probar estos tratamientos, puesto que el beneficio demostrado es escaso y pueden ser costosos, tanto en términos de tiempo, carga para el niño y dinero. Lamentablemente durante mi formación como reumapedia me tocaron varios casos donde padres decidieron abandonar tratamiento, por tratamientos “milagro”, el resultado los niños llegaban peor y en recaída en cuestión de mes o meses.
¿Cuánto tiempo durará la enfermedad?
Tal y como se ha mencionado anteriormente, no existe una cura para el LES. Los signos y síntomas del LES pueden ser mínimos o incluso ausentes si los medicamentos se toman de forma regular tal y como el reumatólogo pediátrico lo ha recetado. Si no se toman los medicamentos de forma regular, las infecciones, el estrés y la luz solar pueden ocasionar el empeoramiento del LES. Este empeoramiento también se conoce como «brote de lupus». Suele ser difícil predecir cuál será la evolución de la enfermedad.
¿Cuál es la evolución a largo plazo (el pronóstico) de la enfermedad?
El desenlace del LES mejora de forma notable cuando se alcanza el control temprano y prolongado de la enfermedad con el uso de la hidroxicloroquina, los corticoesteroides y los Fármacos modificadores de enfermedad. Muchos pacientes con LES de inicio en la infancia tendrán muy buena evolución. Sin embargo, la enfermedad puede ser grave y potencialmente mortal y puede permanecer activa durante la adolescencia y la edad adulta. El pronóstico del LES en la infancia depende del grado de afectación de los órganos internos. Los niños con enfermedad importante de los riñones o del sistema nervioso central necesitan un tratamiento más agresivo. Por otro lado, la erupción cutánea leve y la artritis pueden controlarse con facilidad. Sin embargo, el pronóstico para cada niño es impredecible.
Dra. Nayma Marybel Ruiz Vela
Pediatra sub especialista en reumatología pediátrica
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