Resumo

SALAZAR-VARGAS, Carlos; MONTERROSO-AZOFEIFA, Victoria; ARAUZ-PACHECO, Gloria  e  ROBELO-PENTZKE, Bayardo. Linfangioleiomiomatosis pulmonar. Acta méd. costarric [online]. 2000, vol.42, n.3, pp.134-138. ISSN 0001-6002.

Pulmonary lymphangioleiomyomatosis is an uncommon disease, that affects only women of child-bearing age. The patients present themselves with progressive dyspnea, frequent episodes of spontaneous pneumothorax and, occasionally, with hemoptysis and chylothorax. High resolution CAT sean shows bilateral lung cysts of variable size up to frank bullae. Histologically, numerous thin walied cavities are seen, and dilatation of lymphaties, veins, arteries, bronchioles and alveolar sacs with extensiva smooth muscle proliferation, which engulfs the previously mentioned structures and causes their dilatation. The patients eventually develop respiratory insufficieney and die. This disease is associated with estrogen intake, contraceptivas and becomes worse during pregnancies. Thus, it has been treated with medroxi-progesterone and tamoxifen, with stabilization of the clinical course in some but not in all the cases. We present here the case of a 38 year old female with three episodes of spontaneous pneumothorax and radiological and microscopic documentation of pulmonary lymphangioleiomyomatosis.

Palavras-chave : pneumotórax; disnea; linfangioleiomiomatosis.