CT Differential Diagnosis of Diffuse Cystic Lung Disease by Zonal Predominance

 

Zonal Predominance Diagnosis CT Appearance Clinical Clues / Associations
Upper Lobe Predominant Pulmonary Langerhans Cell Histiocytosis (PLCH) Irregular, bizarre cysts with associated nodules Young adult smokers, mid-upper zone predominance, spontaneous pneumothorax
Centrilobular Emphysema (smoking-related) Lucencies without walls (pseudocysts) Long smoking history, no true cyst walls, upper zone predominance
Sarcoidosis (advanced stage) Fibrotic changes with traction cysts Upper lobe fibrocystic disease, perilymphatic nodules, hilar LAD
Post-TB or granulomatous sequelae Focal thin-walled cavities/cystic change History of TB or fungal infection, calcified granulomas or nodules
Lower Lobe Predominant Alpha-1 Antitrypsin Deficiency (A1ATD) Panacinar emphysema, wall-less lucencies Early-onset emphysema, liver disease, strong lower lobe bias
Lymphocytic Interstitial Pneumonia (LIP) Thin-walled cysts + GGO + septal thickening Sjögren’s, HIV, autoimmune disease
Follicular Bronchiolitis Peribronchial small cysts Often overlaps with LIP, associated with RA or autoimmune disorders
Aspiration/infective sequelae Cystic bronchiectasis History of aspiration, GERD, neuromuscular disease
Diffuse (All Zones) Lymphangioleiomyomatosis (LAM) Uniform, thin-walled round cysts Women of childbearing age, Tuberous Sclerosis, renal angiomyolipoma
Birt-Hogg-Dubé Syndrome (BHD) Basal and subpleural irregular cysts, variable size Skin fibrofolliculomas, renal tumors, spontaneous pneumothorax
Pneumocystis jirovecii Pneumonia (PCP) Ground glass opacities ± small thin-walled cysts Immunocompromised host (esp. HIV/AIDS), ↑ risk of pneumothorax
Amyloidosis Cysts ± nodules ± calcifications Systemic amyloidosis or MALT lymphoma context
Light Chain Deposition Disease (LCDD) Multiple cysts ± nodules Plasma cell dyscrasia, renal dysfunction
Congenital Pulmonary Airway Malformation (CPAM) Multiloculated cysts (may appear diffuse) Pediatric or incidental adult discovery, abnormal developmen