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Antes Revista del Instituto Nacional de Enfermedades Respiratorias

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2006, Número S3

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Neumol Cir Torax 2006; 65 (S3)


Enfermedad pulmonar intersticial en esclerosis sistémica progresiva

Zamora AC, Marmai C, Wolters PJ, Gaxiola M, Navarro C

Idioma: Español
Referencias bibliográficas: 64
Paginas: 4-14
Archivo PDF: 333.87 Kb.


RESUMEN

La escleroderma tiene una prevalencia de 242 casos por millón en los Estados Unidos. Uno de los órganos más afectados es el pulmón, siendo las manifestaciones más frecuentes la enfermedad pulmonar intersticial y la hipertensión arterial pulmonar. La enfermedad intersticial progresa a ser severa en cerca del 15% y hoy en día es la primera causa de muerte en pacientes con escleroderma, con una sobrevida media de 6.5 años después del diagnóstico. Los pacientes en mayor riesgo de desarrollar enfermedad intersticial son aquéllos con el tipo escleroderma difusa y con anticuerpos anti-SCL-70 positivos. En esta revisión, se discuten las manifestaciones radiográficas y patológicas de la fibrosis pulmonar asociada a escleroderma, la utilidad de las pruebas de función pulmonar y del lavado bronquioloalveolar para identificar la presencia de fibrosis y la decisión de a quiénes tratar. Aunque no del todo efectivos, ciclofosfamida o micofenolato ofrecen buena alternativa de tratamiento.


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