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A 52 year old woman with severe chronic respiratory insufficiency caused by pulmonary alveolar microlithiasis was admitted for invasive evaluation, including right and left heart catheterisation, before planned lung transplantation. Right heart catheterisation demonstrated high grade secondary pulmonary hypertension (mean pulmonary artery pressure 51 mm Hg). Coronary angiography did not reveal evidence of atherosclerotic coronary artery disease. However, even with high resolution digital flat panel fluoroscopy, image quality was poor because of diffuse, nodular (“sandstorm-like”) calcifications in both lungs, which are characteristic for pulmonary alveolar microlithiasis.
Pulmonary alveolar microlithiasis is a rare disease of unknown pathogenesis, characterised by widespread intra-alveolar calcium deposits (calci spherites) in the absence of any known disorder of calcium metabolism. It usually occurs in a sporadic form, but also an autosomal recessive form has been described. Patients usually remain asymptomatic for many years. At adult age, however, patients commonly show progressive deterioration of pulmonary function with restrictive pattern, ultimately resulting in respiratory failure associated with cor pulmonale. Lung transplantation is the only effective treatment.
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