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. 2017 Sep 27;26(145):170042. doi: 10.1183/16000617.0042-2017

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.