Figures
- Figure 1.
Case 1. High-resolution computed tomography imaging of the lungs showing intra-alveolar calcifications, mainly subpleural, in posterior and basal parts of the lungs. Paraseptal emphysema is visible. a) Coronal view, b) sagittal view and c and d) axial view of the chest.
- Figure 2.
Case 1. Chest radiograph of the lungs showing bilateral sand-like calcific micronodules <1 mm (sandstorm lung), mainly involving the lower zones (as a result of larger volumina rather than selective predilection).
- Figure 3.
Case 2. High-resolution computed tomography of the lungs showing bilateral calcifications with increased attenuation involving alveoli, intra- and interlobular septa, fissures and pleura. Signs of fibrosis are visible. Hounsfield unit measurements were very high, representing areas with calcifications.
- Figure 4.
Case 1. Lung section from a patient with pulmonary alveolar microlithiasis showing calcified microliths localised in the intra-alveolar spaces. Haematoxylin and eosin staining. Original magnification: a) ×40 and b) ×200.
Tables
- Table 1. Patient characteristics
Case one Case two Sex Male Male Age at diagnosis yrs 50 16 Comorbidity Ischaemic heart disease Nephrolithiasis
Significant haemodynamic aortic valve stenosisHRCT Bilateral lobular, cavitating, nodular infiltrates, ground-glass attenuation Intra- and interlobular septal thickening and consolidation of basal areas
Enlarged lymph nodes in the mediastinum
Paraseptally emphysema
Punctuate calcifications in the lung parenchymaBilateral increased attenuation involving alveoli, intra- and interlobular septa, fissures and pleura
Signs of fibrosis and calcifications
A kidney stonePeripheral O2 without supplemental O2 PFT Normal saturation (98%) Normal saturation (98%) FEV1 L (% pred) 2.97 (72.5) 3.55 (74.6) FVC L 3.61 (71.8) 4.14 (73.3) TLC L 5.22 (70.8) 5.03 (74.1) Diffusing coefficient mmol·min−1·kPa−1·L−1 (% pred) 1.05 (71.7) 4.39 (93.4) Diffusing capacity mmol·min−1·kPa−1 (% pred) 5.36 (49.4) 26.11 (81.8) 6-min walk without oxygen Distance walked m 600 480 Lowest O2 saturation No desaturation No desaturation BAL fluid examination Alveolar macrophages Many giant cells Histopathology Numerous intra-alveolar rounded calcificated bodies with concentrically lamels in the periphery Numerous concentrically calcified concretions
Incrustations and calcifications close to the walls of the alveoliGene mutation in exon 12 of SLC34A2 gene# Homozygous c.1393-1404delACC Homozygous c.1402-1404delACC Treatment Prednisolone/didronate/no treatment Didronate/no treatment HRCT: high-resolution computed tomography; PFT: pulmonary function test; FEV1: forced expiratory volume in 1 s; % pred: % predicted; FVC: forced vital capacity; TLC: total lung capacity; BAL: bronchoalveolar lavage. #: DNA sequencing analysis of whole coding regions of the SLC34A2 (OMIM *604217) gene T chromosome 4P15.31-P15.2 region (reference sequence: NM_006424.2).
