Skip to main content

Table 3 Characteristic clinical manifestations and laboratory tests of DCLD

From: The etiology of diffuse cystic lung diseases: an analysis of 1010 consecutive cases in a LAM clinic

  Clinical manifestations Evaluations
LAM Pneumothorax 35.0%, renal angiomyolipoma 30.2%, chylothorax 17.4%, chylous ascites 3.9%, TSC 8.2% VEGF-D ≥ 800 pg/ml 89.0%
SS Dry mouth 78.9%, dry eyes 65.8% Anti-SSA or anti-SSB antibody positive 97.4%
BHD Pneumothorax 52.2%, fibrofolliculomas or trichodiscomas 17.4% FLCN gene mutation 100%
PLCH Pneumothorax 28.6%, Diabetes insipidus 7.1% MRI showed pituitary funnel lesions 21.4%
  1. BHD Birt–Hogg–Dubé syndrome, LAM lymphangioleiomyomatosis, MRI magnetic resonance imaging, PLCH pulmonary Langerhans cell histiocytosis, SS Sjogren's syndrome, VEGF-D vascular endothelial growth factor-D