Description

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.

 


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