Table 1.

Radiologic features of major DCLD.

Disease	Lung cyst distribution and characteristics	Other radiological features
Lymphangioleomyomatosis (LAM)	•Cysts are regular and thin-walled and usually measure 2-10 mm in diameter •Are diffusely distributed •Vary in number	•Small regular centrilobular nodules •Areas of ground-glass opacity •Pleural effusion •Ascites •Renal angiomyolipoma •Abdominal and pelvic LAM
Pulmonary Langerhans cell histiocytosis (PLCH)	•Cysts are irregular and thick- or thin-walled, later becoming bizarre in shape, and can coalesce •Vary in number and size •Predominate in the upper lung zones, sparing the region of the costophrenic sinuses	•Irregularly marginated centrilobular or peribronchiolar nodules measuring 1-10 mm in diameter •Enlargement of the pulmonary trunk and of the right and left pulmonary arteries
Lymphocytic interstitial pneumonia (LIP)	•Cysts vary in shape •thin-walled •Measure up to 30 mm in diameter •Are diffusely distributed, predominating in the lower lobes and along the peribronchovascular bundle	•Ground-glass opacities and focal consolidations •Peribronchovascular bundle thickening •Poorly defined centrilobular nodules •Interlobular septal thickening •Reticular opacities •Mediastinal and hilar lymph node enlargement
Follicular bronchiolitis	•Cysts vary in shape and are thin-walled •Measure up to 30 mm in diameter •Are diffusely distributed, predominating in the lower lobes and along the peribronchovascular bundle	•Centrilobular nodules or ground-glass opacities
Birt-Hogg-Dubé syndrome (BHD)	•Cysts are multiple and irregular - Predominate in the lower medial and subpleural regions of the lung
Desquamative interstitial pneumonia (DIP)	•Cysts are regular and measure up to 20 mm in diameter •Predominate in the lower lung zones	•Diffuse ground-glass opacities predominating in the lower lung zones - Mild distortion of the lung architecture