Typical pulmonary manifestation of lymphangioleiomyomatosis (LAM). The first symptomatic presentation of these patients may be a pneumothorax.
However, the diagnosis of LAM should not be based on pulmonary CT features alone. One or more of the following features should be present: tuberous sclerosis complex, angiomyolipomas of the kidneys, elevated VEGF-D levels, a chylous pleural effusion or a lung biopsy for the histological evaluation of LAM 1.
In this case, renal angiomyolipomas were histologically confirmed before the initiation of immunosuppressive therapy.
Summarized, when seeing typical pulmonary cystic changes, the kidneys should be checked for renal angiomyolipomas, and the brain should be checked for tubers, migrational lines and subependymal giant cell astrocytomas (SEGA).