Médecine pulmonaire et respiratoire

Misdiagnosing Bronchiectasis and Lymphangioleiomyomatosis

Abstract

Malihe Ghadir and Katayoon Najafizadeh

This report presents a 25 year old female with a history of exertional dyspnea since the age of three. She was wrongly diagnosed and managed as bronchiectasis although she had had no productive cough or recurrent infection. At the age of 23, because of her dyspnea she was referred to a pneumologist for the first time. High resolution computed tomography and open lung biopsy was performed at that time. High resolution computed tomography showed diffuse thin walled cysts and pathologic results also confirmed lymphangioleiomyomatosis as her definite diagnose. She was treated with sirolimus and progesterone for two years which resulted in significant increase in oxygen saturation. However, no change was seen in forced expiratory volume at first second.