Criteria for a definite diagnosis of lymphangioleiomyomatosis according to guidelines
| ERS guidelines 2010 | ATS/JRS guidelines 2016 |
| Characteristic HRCT scans# and one of the following: | |
| Tuberous sclerosis complex | Tuberous sclerosis complex |
| Chylous effusions | Chylous effusions |
| Angiomyolipomas | Angiomyolipomas |
| Lymphatic involvement | Lymphatic involvement |
| Serum VEGF-D ≥800 pg·mL−1 | |
#: numerous (more than 10) thin-walled lung cysts distributed diffusely throughout the lungs without sparing of lung bases, without any other significant pulmonary involvement (except for multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis complex). ERS: European Respiratory Society; ATS/JRS: American Thoracic Society/Japanese Respiratory Society; HRCT: high-resolution computed tomography; VEGF-D: vascular endothelial growth factor-D.