Pulmonary involvement is seen exclusively in woman over the age of 30 and is quite rare (1% of the women with TSC). Two forms have been described: pulmonary cysts and lymphangioleiomyomatosis (LAM). Cysts can remain silent or rupture causing pneumothorax, shortness of breath and hemoptysis. LAM is more insidious causing progressive restrictive lung disease. Lung transplantation has been used with varying degrees of success.