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Pages 85-93 (January 2011)
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Vol. 47. Issue 2.
Pages 85-93 (January 2011)
Review Article
DOI: 10.1016/S1579-2129(11)70019-2
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Lymphangioleiomyomatosis
Linfangioleiomiomatosis
Visits
...
Emilio Ansótegui Barreraa, Nuria Mancheño Franchb, Francisco Vera-Sempereb, José Padilla Alarcónc,??
Corresponding author
jpadilla@comv.es

Corresponding author.
a Servicio de Neumología, Hospital Universitario La Fe, Valencia, Spain
b Servicio de Anatomía Patológica, Hospital Universitario La Fe, Valencia, Spain
c Servicio de Cirugía Torácica, Instituto Valenciano de Oncología, Valencia, Spain
Article information
Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease that mainly affects women, particularly those of fertile age. Its presentation is sporadic or associated with tuberous sclerosis complex. It is characterized by an abnormal proliferation of immature smooth muscle cells (LAM cells), which grow aberrantly in the airway, parenchyma, lymph nodes and pulmonary blood vessels and can gradually lead to respiratory failure. It affects several systems, affecting the lymphatic ganglia and causing abdominal tumors. Given its very low prevalence, difficulty in establishing early diagnosis, absence of curative treatment and the difficulty in obtaining information, LAM is placed under the heading of the so-called Rare Diseases. There is a growing interest in the study of this disease which has led to the creation of patient registers and an exponential growth in LAM research, both at a clinical and cellular level.

Keywords:
Lymphangioleiomyomatosis
Tuberous sclerosis
Diffuse lung diseases
Resumen

La linfangioleiomiomatosis (LAM) es una enfermedad rara que afecta predominantemente a la mujer, sobre todo en edad fértil. Se presenta de forma esporádica o bien asociada al complejo de esclerosis tuberosa. Se caracteriza por una proliferación anormal de células musculares lisas inmaduras (células LAM), que crecen de manera aberrante en la vía aérea, parénquima, linfáticos y vasos sanguíneos pulmonares, lo que determina una evolución progresiva hacia la insuficiencia respiratoria. Tiene carácter multisistémico, afectando a ganglios linfáticos y produciendo tumores abdominales. Dadas su escasa prevalencia, la dificultad de establecer un diagnóstico precoz, la ausencia de un tratamiento curativo y la dificultad de obtener información encuadran a la LAM dentro del capítulo de las denominadas Enfermedades Raras. Existe un creciente interés en el estudio de esta enfermedad, lo que ha determinado el establecimiento de registros de pacientes y un crecimiento exponencial en la investigación de la LAM, tanto a nivel clínico como celular.

Palabras clave:
Linfangioleiomiomatosis
Esclerosis tuberosa
Neumopatías difusas
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