Pulmonary lymphoangioleiomyomatosis (LAM) is a rare destructive lung disease typically
affecting women of childbearing age. Currently, there is no effective therapy for the
disease and the prognosis is poor.
In order to better study this disease, the National Heart, Lung, and Blood Institute (NHLBI)
has developed a registry to keep an official record of patients diagnosed with LAM. This
research project will collect data from 6 health care centers as well as outside physicians.
Researchers hope to provide valuable information about the rate of lung destruction and
quality of life in patients with LAM.
Patients participating in this study will be followed for 5 years. Tissue collected from
these patients may contribute to the development of future studies on the disease processes
LAM is a rare disease that predominantly affects women of child-bearing age. To study in
more detail this rare disease, this multi-center project will establish a registry of
persons with LAM. By combining data from 6 centers and outside physicians, this study may
yield valuable information regarding the rate of decline in pulmonary function and quality
of life in individuals with LAM. These patients will be followed over a five-year period.
Tissue collected from study participants may facilitate future studies into the molecular
basis of LAM.
Informed consent must be obtained from the patient.
Patients must be female.
Patients must be age 18 or older.
Patients with prevalent and incident cases are eligible.
Patients with the presence or absence of underlying diagnosis or evidence of Tuberous
Sclerosis Complex (TSC) are eligible.
Patients must have a diagnosis of LAM confirmed by any of the following criteria:
Lung biopsy (transbronchial, surgical, transthoracic) judged to be diagnostic by the
Tissue Core pathologists;
Biopsy of lymph node or other mass judged to be diagnostic by the Tissue Core
High resolution CT scan of the chest which is judged to be diagnostic of LAM with a high
degree of certainty by all three of the expert radiologists making up the Imaging Core.
Patients may be enrolled in other protocols.