000 Alveolar Proteinosis

Position of Disease
Perihilar distribution
Ashley Davidoff MD TheCommonvein.net lungs-0770
Accumulation of proteinaceous material in the alveoli, impairing gas exchange and leading to respiratory failure. Extensive thickening of interlobular septa leading to crazy paving appearance
Ashley Davidoff TheCommonVein.net
lungs-0738b

 

Alveolar proteinosis in a 37-year-old woman with a nonproductive cough and dyspnea. (a) Posteroanterior chest radiograph shows bilateral reticular areas of increased opacity, which occur predominantly in the lower
zones. (b) High-resolution CT scan shows diffuse geographic ground-glass attenuation with superimposed intra- and
interlobular septal thickening (arrowhead). Note the polygonal appearance, which represents the secondary pulmonary lobule. (c, d) Photomicrographs (original magnification, 400; hematoxylin-eosin [c] and periodic acid–Schiff
[d] stains) of a specimen from transbronchial biopsy show alveolar spaces filled by a dense, eosinophilic, granular
proteinaceous material () that is positive for periodic acid–Schiff stain.
Rossi, S.E et al “Crazy-Paving” Pattern at Thin-Section CT of the Lungs: RadiologicPathologic Overview Radiographics  Volume 23 – Number 6,  2003
48 year old male with alveolar proteinosis
Ashley Davidoff TheCommonVein.net
48 year old male with alveolar proteinosis-
central distribution
Ashley Davidoff
TheCommonVein.net

Follow up

48 year old male with alveolar proteinosis
Follow up
Ashley Davidoff
TheCommonVein.net

 

A Second Patient

35 year old female with alveolar proteinosis
Excellent example of crazy paving
Ashley Davidoff TheCommonVein.net 117625

 

Crazy-paving sign. Axial CT of the chest shows thickening of the intralobular and interlobular septa with a superimposed background of ground-glass opacity in a patient with pulmonary alveolar proteinosis.
Source
Signs in Thoracic Imaging
Journal of Thoracic Imaging 21(1):76-90, March 2006.
  • Alveolar proteinosis is a rare lung disease.
  • It involves the accumulation of proteinaceous material in the alveoli, impairing gas exchange and leading to respiratory failure.
  • There are three types: primary, secondary, and congenital.
  • Symptoms include shortness of breath, cough, fatigue, weight loss, and chest pain.
  • Diagnosis is typically made through imaging studies
      • Diffuse bilateral ground-glass opacities
      • “Crazy-paving” appearance
      • Thickened interlobular septa and intralobular lines superimposed on ground-glass opacities
      • Consolidation may be present, particularly in advanced cases
      • Homogeneous opacities involving the upper lobes in primary alveolar proteinosis
      • Patchy opacities with peripheral distribution in secondary alveolar proteinosis
      • CT findings are non-specific and can resemble other lung diseases, so biopsy is often necessary for diagnosis.
    • and a lung tissue biopsy.
  • Treatment may involve whole-lung lavage, medications, or lung transplantation.