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The Calcareous Lung

      An 84-year-old woman, who was a nonsmoker, was admitted for aortic valve replacement. Chest radiography and preoperative computed tomography (CT) of the lungs revealed diffuse interstitial lung disease with calcification seen on the pleural lining and throughout the lungs. Two of the patient's sisters had the same lung findings and died at an early age due to pulmonary diseases.
      Diagnosis of pulmonary alveolar microlithiasis (PAM) was confirmed in the patient by the presence of microliths in the bronchoalveolar lavage sample. Bioprosthetic valve replacement for aortic stenosis was successful.
      PAM is characterized by diffuse calcifications within the alveoli. The disease is more prevalent in Europe (37% of all reported cases) than in the United States (8% of all reported cases). Family history was present in 54% of cases, and female sex was predominant in familial cases. A standard radiograph with a family history of PAM is sufficient to make the diagnosis.
      • Castellana G
      • Lamorgese V
      Pulmonary alveolar microlithiasis:world cases and review of the literature.
      Characteristic chest radiographic appearance of PAM is bilateral infiltrates with fine, sandlike calcium phosphate inclusions involving mostly the middle and lower zones. A CT scan of the lungs reveals diffuse ground-glass opacities, with calcification along interlobular septa.
      • Marchiori E
      • Gonçalves CM
      • Escuissato DL
      • et al.
      Pulmonary alveolar microlithiasis: high-resolution computed tomography findings in 10 patients.
      A CT scan followed by a bronchoalveolar lavage sample showing microliths or uptake of technetium Tc 99m diphosphonate within the lungs may confirm the diagnosis. If characteristic radiological findings are present, lung biopsy is unnecessary except in patients with unusual presentation.
      • Gasparetto EL
      • Tazoniero P
      • Escuissato DL
      • et al.
      Pulmonary alveolar microlithiasis presenting with crazy-paving pattern on high resolution CT.
      Differential diagnoses include almost all interstial lung diseases, with specific attention to miliary tuberculosis, fungal pneumonia, and pulmonary alveolar proteinosis.
      • Thapa R
      • Ganguly D
      • Ghosh A
      Pulmonary alveolar microlithiasis in siblings.
      Currently, bilateral lung transplant is the only effective treatment available. However, the disease may recur in the transplanted lungs. Patients often develop pulmonary hypertension that leads to death.
      • Shigemura N
      • Bermudez C
      • Hattler BG
      • et al.
      Lung transplantation for pulmonary alveolar microlithiasis.

      REFERENCES

        • Castellana G
        • Lamorgese V
        Pulmonary alveolar microlithiasis:world cases and review of the literature.
        Respiration. 2003; 70: 549-555
        • Marchiori E
        • Gonçalves CM
        • Escuissato DL
        • et al.
        Pulmonary alveolar microlithiasis: high-resolution computed tomography findings in 10 patients.
        J Bras Pneumol. 2007; 33: 552-557
        • Gasparetto EL
        • Tazoniero P
        • Escuissato DL
        • et al.
        Pulmonary alveolar microlithiasis presenting with crazy-paving pattern on high resolution CT.
        Br J Radiol. 2004; 77: 974-976
        • Thapa R
        • Ganguly D
        • Ghosh A
        Pulmonary alveolar microlithiasis in siblings.
        Indian Pediatr. 2008; 45: 154-156
        • Shigemura N
        • Bermudez C
        • Hattler BG
        • et al.
        Lung transplantation for pulmonary alveolar microlithiasis.
        J Thorac Cardiovasc Surg. 2010; 139: e50-e52