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Pulmonary alveolar microlithiasis: review and management
Teruo Tachibana1, Koichi Hagiwara, Takeshi Johkoh
1Department of Internal Medicine, Aizenbashi Hospital, Osaka, Japan.
Current Opinion in Pulmonary Medicine
|July 21, 2009
Summary
Pulmonary alveolar microlithiasis (PAM) is linked to SLC34A2 gene mutations. Updated research details imaging, genetics, and poor prognosis for this rare lung disease.
Area of Science:
- Pulmonary Medicine
- Genetics
- Radiology
Background:
- Pulmonary alveolar microlithiasis (PAM) is a rare lung disease characterized by diffuse calcifications.
- Recent advancements have expanded understanding since its initial description.
Purpose of the Study:
- To provide updated information on the clinical course, imaging findings, and genetics of PAM.
- To correlate genetic findings with disease pathogenesis and clinical presentation.
Main Methods:
- Review of updated literature on PAM.
- Analysis of chest computed tomography (CT) findings and their correlation with pathological data.
- Evaluation of serum surfactant protein levels as potential biomarkers.
- Assessment of long-term follow-up data.
Main Results:
- The SLC34A2 gene, encoding a sodium-dependent phosphate transporter, has been identified as causative for PAM.
- Characteristic chest CT findings include perilobular interstitial thickening and calcification, correlating with pathology.
- Elevated serum surfactant proteins A and D are associated with disease progression.
Conclusions:
- Mutation in SLC34A2 confirms PAM diagnosis and offers insight into pathogenesis.
- Specific CT findings and serum biomarkers aid in diagnosis and monitoring.
- PAM has a poor prognosis, highlighting the urgent need for effective treatments.
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