Presentation
Progressive severe dyspnea. Never-smoker.
Patient Data
Age: 45 years
Gender: Female
From the case:
Sporadic lymphangioleiomyomatosis
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Large lungs.
Innumerable scattered thin-walled cysts.
Small areas of normal lung at the apices and bases highlighted by the MinIPs.
Case Discussion
The diagnosis was established by the characteristic CT appearances and elevated blood VEGF-D. Her kidneys were normal and there were no effusions or lymphatic masses.
When cystic lung disease is advanced, the appearance may be mistaken for emphysema as the cyst walls may be almost imperceptible, especially in areas where there is no normal intervening lung.
Treatment: sirolimus typically slows disease progression, bronchodilators, home oxygen for respiratory failure, lung transplantation for severe lung disease.