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% |