PULMONARY ALVEOLAR MICROLITHIASIS - REVIEW OF ITALIAN REPORTS

Pulmonary alveolar microlithiasis (PAM) is a rare disease of unknown e tiology, characterized by the presence of calcific concretions in the alveolar spaces. A familial occurrence is frequently found so that an inherited trait is thought to be involved. The chest X-ray is characte rized by a 'sandstorm' picture while the clinical state undergoes to a slow and progressive impairment resulting in respiratory failure at t he end stage. We have reviewed the Italian literature of the past 50 y ears detecting 48 case-reports of PAM (19 males and 29 females). Only 20 out of them were documented in international journals. A familial o ccurence of 43.7% was found and 18 patients were under age fifteen; Th ere was a prevalence in the female sex (60.4%) and in the second decad e of life. Chest X-ray was the most important tool to diagnose PAM rev ealing the characteristic picture in all patient. Bronchoalveolar lava ge (BAL) and open lung biopsy respectively show the characteristic cal cospherites in the recovered fluid (BALF) and in the alveolar spaces. About 300 cases of PAM are reported in the international literature. W e believe these data are probably underestimated because many case-rep orts are not published in international literature.