Pulmonary alveolar microlithiasis and interstitial pneumonitis: a case report of the west of Iran

Pulmonary alveolar microlithiasis (PAM) is a rare disease with autosomal recessive inheritance. Herein, a 20-year-old lady referred to the hospital with a dry cough for two years. The chest X-ray findings were bilateral reticulonodular opacities in both lungs and honeycomb appearance suspicious for miliary tuberculosis and idiopathic pulmonary fibrosis. A wedge biopsy of lung showed that there were several intraalveolar laminated concretions in the pathology report compatible with pulmonary alveolar microlithiasis and interstitial infiltration of lymphocytes and neutrophils compatible with interstitial pneumonitis. PAM is a rare progressive disease with the production of microliths in pulmonary alveoli. The pathologist, radiologist, and clinician should be familiar with this entity for diagnosis and appropriate management. The family of the patient especially siblings must be evaluated for earlier diagnosis.