TABLE 1.

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.