Pulmonary Alveolar Microlithiasis: An Indolent Disease with Astounding Radiological Findings

Abstract

Pulmonary Alveolar Microlithiasis is a disorder in which calcospherites composed of calcium and phosphorous accumulate in the alveoli throughout the lung parenchyma leading to a progressive infiltrative lung disease. It can present completely asymptomatically in early childhood, only to end with cor pulmonale in later life. We report two cases - the first, a six - year - old boy who had a strong familial history of lung diseases and presented with a history of chronic cough. No further symptoms were elicited, although two elder familial members had passed away of cor pulmonale in their mid - 30s. The other is an eight - year - old boy referred to as a case of “ non - responsive pulmonary tuberculosis, diagnosed sans microbiological evidence. Respiratory examination, and growth were normal in both children. Chest X - ray had a typical ‘ Stone Lung ’ appearance. The clinical findings were in contrast to the radiological picture and hence a CT scan (Chest) was done. On further suspicion, lung biopsy and genetic analysis established the diagnosis of Pulmonary Alveolar Micro-lithiasis The patients were given supportive treatment and started on oral Etidronate. With an indolent clinical course, Pulmonary Alveolar Microlithiasis poises itself to remain a “ missed diagnosis ” with eventual poor outcome, unless the paediatrician is vary about the astounding radiological findings, which should immediately prompt towards further workup for the disease.