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Colegio de Medicina Interna de México.

2006, Número 3

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Med Int Mex 2006; 22 (3)


Esclerosis sistémica

Vera LOL

Idioma: Español
Referencias bibliográficas: 99
Paginas: 231-245
Archivo PDF: 373.82 Kb.


RESUMEN

La esclerosis sistémica o esclerodermia es una enfermedad del tejido conjuntivo, de causa desconocida, que se distingue por excesiva producción de la matriz extracelular (colágeno), que produce fibrosis en la piel y en algunos órganos internos, así como por daño del endotelio de los vasos pequeños con isquemia tisular y por alteraciones inmunológicas. La patogénesis comienza con un estímulo ambiental en un individuo predispuesto genéticamente; éste activa al sistema inmunitario, con liberación de mediadores inmunológicos (citocinas), que producen daño endotelial, proliferación de fibroblastos y síntesis de colágeno. La liberación de citocinas endoteliales promueve la mayor activación del sistema inmunológico. La esclerosis sistémica se clasifica en esclerosis sistémica difusa, sistémica limitada y esclerodermia sin esclerodermia. La esclerosis sistémica difusa se distingue por el endurecimiento generalizado de la piel y la sistémica limitada, por el endurecimiento de la piel que afecta las extremidades distales, la cara y el cuello, el síndrome de CREST es una forma limitada de esta enfermedad; en la esclerodermia sin esclerodermia la piel no está afectada, sólo existe daño de los órganos. La esclerosis sistémica se distingue por tener afectación multisistémica y crónica. Las manifestaciones clínicas iniciales más frecuentes son el fenómeno de Raynaud, la fatiga y las alteraciones musculoesqueléticas. La piel es el órgano blanco implicado en esta enfermedad y su afección se distingue por tres fases: 1) fase edematosa, 2) fase de induración, y 3) fase atrófica. El tubo digestivo es otro de los sistemas más afectados, y de éste el esófago. El daño musculoesquelético se manifiesta por dolores articulares, contracturas en flexión por fibrosis de los tendones y otras. La lesión pulmonar más característica es la fibrosis pulmonar, aunque puede existir hipertensión arterial pulmonar; el corazón también se daña y puede haber arritmias, disfunción del ventrículo izquierdo, insuficiencia cardiaca, entre otras. El riñón también se ve afectado y puede ocasionar crisis renal y daño tubulointersticial. Los criterios diagnósticos son la esclerodermia proximal más dos de los siguientes criterios menores: esclerodactilia, cicatrices digitales, pérdida de tejido en los pulpejos de los dedos y fibrosis pulmonar bibasal. Para su tratamiento se han administrado varios medicamentos, cuyos resultados han sido desalentadores. La D-penicilamina es un antifibrótico efectivo en el manejo de esta enfermedad. Sin embargo, hay algunos adelantos en el tratamiento del fenómeno de Raynaud, como los antagonistas del calcio (nifedipina); los procinéticos e inhibidores de la bomba de protones para las manifestaciones gastrointestinales; los pulsos de metilprednisolona y la ciclofosfamida para la fibrosis pulmonar, y los pulsos de ciclofosfamida y el antagonista de los receptores de la endotelina, bosentán, para la hipertensión arterial pulmonar.


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