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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