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Table 1 Summary of metabolic lung diseases

From: Metabolic and storage lung diseases: spectrum of imaging appearances

Disease Aetiology Pathology Relevant clinical features Imaging findings
Pulmonary calcification Dystrophic or metastatic mechanisms Deposition of calcium in the alveolar septa Often asymptomatic Usually non-diagnostic Numerous subcentimetre calcified nodules
Pulmonary ossification
 Nodular form Chronic passive congestion Calcified/ossified intra-alveolar masses Often asymptomatic Usually non-diagnostic Mid to lower lung predominant 1-5 mm calcified nodules
 Dendriform form Unclear, occurs in areas of chronic lung insult Branching calcifications affecting the interstitium Often asymptomatic Serpentine opacities Characteristic lower lobe predominant branching delicate calcifications, often in areas of fibrosis
Pulmonary alveolar microlithiasis Unclear, association with SLC34A2 gene Intra-alveolar deposition of microliths Often asymptomatic, occurs in all age groups Diffuse small calcified nodules, giving a “sandstorm” appearance. Lucent subpleural line Diffuse uniform calcified micronodules with a lower lung predominance
Pulmonary amyloidosis Extracellular deposition of insoluble beta pleated proteins   All age groups   
 Tracheobronchial form   Involvement of trachea and airways Upper airway obstruction, stridor, dyspnea Narrowing of the tracheal lumen Nodules, plaques or wall thickening of the subepithelial trachea, which may be calcified
 Nodular parenchymal form   Lung nodule/mass Asymptomatic Peripheral solitary or multiple nodules Peripheral well circumscribed nodule/s which may be calcified
 Diffuse interstitial form   Amyloid infiltration of pulmonary interstitium Dyspnea Reticular markings Interlobular septal thickening, which may be nodular; calcifications may be seen
Pulmonary alveolar proteinosis Mutations in surfactant protein or GM-CSF genes Intra-alveolar accumulation of surfactant like material Young to middle aged men, modest clinical symptoms Drastic appearance with bilateral perihilar airspace disease Interlobular septal thickening with ground-glass opacities giving a “crazy paving” appearance
  1. CXR chest radiography, CT computed tomography, GM-CSF granulocyte monocyte-colony stimulating factor