The European Respiratory Society (ERS) has published diagnostic criteria for lymphangioleiomyomatosis (LAM).
Parameters:
(1) clinical history compatible with LAM
(2) high resolution computed tomography (HRCT) of the lungs
(3) tuberous sclerosis complex (TSC)
(4) renal angiomyolipoma
(5) thoracic and/or abdominal chylous effusion
(6) lymphangioleiomyomatosis (LAM) in a lung biopsy
(7) lymphangioleiomyoma (LAM) not in lung
Pulmonary HRCT |
Other Finding |
LAM in Female |
characteristic |
LAM in lung biopsy |
definite |
compatible |
LAM in lung biopsy |
definite |
characteristic |
renal angiomyolipoma |
definite |
characteristic |
thoracic or abdominal chylous effusion |
definite |
characteristic |
LAM other than lung |
definite |
characteristic |
TSC (definite or probable) |
definite |
characteristic |
clinical history |
probable |
compatible |
renal angiomyolipoma |
probable |
compatible |
thoracic or abdominal chylous effusion |
probable |
characteristic |
NA |
possible |
compatible |
NA |
possible |
Evidence TSC |
Pulmonary HRCT |
Other Finding |
LAM in Male |
present |
characteristic |
LAM in lung biopsy |
definite (exceptional) |
present |
compatible |
LAM in lung biopsy |
definite (exceptional) |
absent |
characteristic |
LAM in lung biopsy |
very exceptional |
The diagnosis of LAM requires exclusion of alternative causes of cystic lung disease.
Specialty: Hematology Oncology, Surgery, general