Pulmonary alveolar microlithiasis

Last revised by Henry Knipe on 29 Dec 2023

Pulmonary alveolar microlithiasis is a rare idiopathic condition characterized by widespread intra-alveolar deposition of spherical calcium phosphate microliths (calcospherites).

A slight female predilection may be present in the familial form 2. Most cases are reported in Asia and Europe 9.

Often discovered incidentally on a chest radiograph. The radiographic features are frequently disproportionate to the clinical symptoms 5.

Pulmonary alveolar microlithiasis is believed to be due to a mutation in the SLC34A2 gene that causes inactivation of a sodium-dependent phosphate cotransporter, which is found mainly in alveolar type II cells. This cotransporter normally clears phosphate from degraded surfactant, and when inactivated there is accumulation of phosphate in the alveolus, and calcium phosphate microliths are then thought to form 9

An autosomal recessive inheritance pattern has been proposed given familial occurrence in a majority of cases 2,3,9. Usually, there is no abnormal calcium metabolism.

The distribution of pulmonary alveolar microlithiasis is bilateral with a middle and lower zone predilection 2,3.

The pattern in children differs from that in adults 2.

Chest x-ray typically demonstrates:

HRCT better shows numerous sand-like calcifications throughout the lungs with subpleural and peribronchial distribution (typically ~1 mm) 8. Additional accompanying HRCT features include

On occasion, PET may show high FDG uptake, but primarily in the lung regions that spare calcification 10.

No known treatment is available. Overall prognosis is good, but occasionally slow progression can result in an end-stage lung fibrosis requiring lung transplantation. 

Some consider the radiographic appearance to be pathognomonic 3.

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