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Revista chilena de enfermedades respiratorias

On-line version ISSN 0717-7348

Abstract

PINEDA-BOCANEGRA, José L.; VELASQUEZ-CANTILLO, Katya Lissette; MAESTRE-SERRANO, Ronald  and  SANTIAGO-HENRIQUEZ, Ernesto Agustín. Pulmonary lymphangioleiomyomatosis, a rare lung disease: presentation of a clinical case. Rev. chil. enferm. respir. [online]. 2019, vol.35, n.1, pp.58-62. ISSN 0717-7348.  http://dx.doi.org/10.4067/S0717-73482019000100058.

Introduction:

Pulmonary lymphangioleiomyomatosis (LAM) is a rare and progressive disease; characterized by airway, lymphatic and blood vessels-smooth muscle cells excessive proliferation. Added to the abnormal cell growth, parenchymal cystic degeneration is present, which can be reflected initially as a asymptomatic course and can progress to severe gaseous exchange deterioration and fulminating respiratory insufficiency.

Case description:

A 41-year-old female patient with a clinical course consisting of occasional dry cough, associated with mild pleuritic pain on the right side of thorax. As no improvement was achieved, thoracic imaging study was performed, where a right pneumothorax was found. Tomography images showed multiple lung cystic lesions. Anatomopathological study reports structural changes compatible with LAM.

Conclusion:

Given the simplicity of the symptoms that LAM can debut with, its diagnostic confirmation is generated in advanced stages of the disease, when the important pulmonary damage leads to the appearance of clinical factors with greater impact on the general state of patients so early thoracic imaging studies gain vital importance.

Keywords : Lymphangioleiomyomatosis; pneumothorax; tomography, X-Ray computed; lung; myocytes, smooth muscle.

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