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Linfangioleiomiomatosis esporádica. Dos casos clínicos
Resumen
La linfangioleiomiomatosis (LAM) es una enfermedad pulmonar rara, de etiología desconocida, descrita desde 1918 asociada con complejo de esclerosis tuberosa; posteriormente se informaron formas esporádicas. Se incluye entre las enfermedades pulmonares intersticiales difusas. El objetivo del presente artículo es evaluar la presentación clínica, evolución y manejo en dos pacientes, así como hacer una revisión de actualidades terapéuticas. Las pacientes que se describen se presentaron con cuadro de neumotórax espontáneo. Su estudio tomográfico mostró lesiones quísticas características. Se les realizó toracotomía con toma de biopsia pulmonar por no tener reexpansión pulmonar a pesar de sonda endopleural. Aunque existe controversia sobre el manejo óptimo, en pacientes con rápido deterioro de la función respiratoria se avala el uso de progesterona por al menos 12 meses. Debido a lo infrecuente de la enfermedad, se requiere un registro de pacientes para evaluar el uso de fármacos experimentales o la inclusión en protocolos de investigación a fin de mejorar su pronóstico.
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