Pulmonary alveolar microlithiasis complicated by corpulmonale

G G A Gayani,M R Mohideen,A Perera,C K Bodinayake,S Muhunthan

doi:10.4038/jccp.v44i1-2.7691

Abstract

Pulmonary alveolar microlithiasis is a rare,chronic lung disease with bilateral intra-alveolar calcium and phosphate deposition throughout the lung parenchyma. This is a case history of a 40- year-old female with a family history of lung disease, presenting with progressive respiratory symptoms for 5 years. She had clinical and radiological features of pulmonary alveolar microlithiasis and responded poorly to medical treatment, subsequently admitted with severe respiratory insufficiency, pulmonary hypertensionand right heart failure. She died after repeated attempts at resuscitation. Her autopsy revealed atherosclerotic pulmonary arteries with intra alveolar deposits of concentrically laminated calcific bodies which confirmed the diagnosis of pulmonary alveolar microlithiasis. The disease evolved in a progressive protracted course with respiratory insufficiency which led to corpulmonale. DOI: http://dx.doi.org/10.4038/jccp.v44i1-2.7691 Journal of the Ceylon College of Physicians, 2013, 44, 40-41

Highlights

Pulmonary alveolar microlithiasis is a rare pulmonary disease of unknown pathogenesis characterized by widespread laminated calcipherites in alveolar spaces in the absence of any known disorder of calcium metabolism. It usually occurs in sporadic form, and tend to occur in families with autosomal recessive pattern of inheritance
Very few cases have been reported in Sri Lanka[1,2]
High resolution CT of the chest revealed fine nodular calcific densities scattered throughout both lung fields along the peribronchiolar, subpleural and mediastinal pleural aspects suggestive of pulmonary alveolar microlithiasis