TABLE 3

Diagnostic criteria for lymphangioleiomyomatosis (LAM)

Compatible clinical history
AND
Characteristic HRCT of the chest
AND

  • One or more of:

  •  Presence of TSC

  •  Renal angiomyolipoma(s)

  •  Elevated serum VEGF-D >800 pg·mL−1

  •  Chylous effusion (pleural or ascites) confirmed by tap and biochemical analysis of the fluid

  •  Lymphangioleiomyomas (lymphangiomyomas)

  •  Demonstration of LAM cells or LAM cell clusters on cytological examination of effusions or lymph nodes

  •  Histopathological confirmation of LAM by lung biopsy or biopsy of retroperitoneal or pelvic masses

HRCT: high-resolution computed tomography; TSC: tuberous sclerosis complex; VEGF: vascular endothelial growth factor. Reproduced and modified from [93] with permission.