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Pulmonary alveolar microlithiasis. State-of-the-art review

Flávia Angélica Ferreira Francisco1, Jorge Luiz Pereira e Silva, Bruno Hochhegger

  • 1Department of Radiology, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil. flaviangel@gmail.com

Respiratory Medicine
|November 28, 2012
PubMed

Insights

Pulmonary alveolar microlithiasis (PAM) is a rare genetic lung disease caused by SLC34A2 gene mutations. This review covers its pathology, clinical features, and imaging, highlighting the poor prognosis and lack of current treatments.

Area of Science:

  • Pulmonary Medicine
  • Genetics
  • Radiology

Background:

  • Pulmonary alveolar microlithiasis (PAM) is a rare genetic disorder.
  • Characterized by diffuse calcifications within the lung alveoli.
  • Caused by mutations in the SLC34A2 gene, affecting phosphate transport.

Purpose of the Study:

  • To review the key pathological features of PAM.
  • To describe the clinical presentation and diagnostic imaging findings.
  • To discuss the genetic basis and current treatment landscape.

Main Methods:

  • Literature review of pathological, clinical, and imaging studies on PAM.
  • Analysis of genetic data related to SLC34A2 mutations.
  • Synthesis of information on disease progression and prognosis.

Main Results:

  • PAM involves intra-alveolar phosphate accumulation leading to microlith formation.
  • Clinical-radiological dissociation is a hallmark feature.
  • The disease has a poor long-term prognosis with no established treatments.

Conclusions:

  • PAM is a rare genetic lung disease with distinct pathological and imaging findings.
  • Understanding the genetic basis is crucial for diagnosis.
  • Further research is needed for effective therapeutic strategies.

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