DOI: https://doi.org/10.36347/sasjm.2026.v12i04.023

Pages: 362-366

Pulmonary alveolar microlithiasis (PAM) is an ultra-rare diffuse lung disease caused by biallelic pathogenic variants in the SLC34A2 gene, resulting in defective phosphate clearance by type II pneumocytes and progressive intra-alveolar deposition of calcium phosphate microliths. The disease classically evolves with clinico-radiological dissociation: radiographic abnormalities may be striking long before severe respiratory symptoms appear. We report the case of a 42-year-old Moroccan man with diabetes who presented with gradually progressive exertional dyspnea over several months. Chest radiography revealed diffuse bilateral micronodular opacities resembling a sandstorm pattern. Contrast-enhanced chest computed tomography demonstrated innumerable bilateral calcified micronodules with predominantly subpleural and peribronchovascular distribution, associated with bilateral subpleural cystic lesions and paraseptal emphysema. The examination also showed dilation of the main pulmonary artery, enlargement of the right-sided cardiac chambers, a right-to-left ventricular ratio greater than 1, and moderate pericardial effusion, raising strong suspicion for pulmonary hypertension complicating advanced PAM. This report highlights the decisive role of thoracic imaging in suggesting the diagnosis, especially in settings where genetic or histopathological confirmation may not be immediately available. The discussion places the present case in the context of the largest available reviews and recent case reports, with emphasis on pathophysiology, characteristic imaging findings, functional decline, major complications such as pulmonary hypertension and pneumothorax, differential diagnosis, and current treatment limitations.