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Refining the Radiologic Recognition of Pulmonary Alveolar Proteinosis: A Crazy-Paving Pattern Approach.

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Pulmonary alveolar proteinosis (PAP) is a rare lung disease. This review uses a phenotype-first approach to differentiate PAP from other conditions presenting with the crazy-paving pattern on HRCT scans.

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Area of Science:

  • Radiology
  • Pulmonology
  • Pathology

Background:

  • Pulmonary alveolar proteinosis (PAP) is a rare airspace disease.
  • The "crazy-paving" pattern on high-resolution computed tomography (HRCT) is suggestive but not pathognomonic of PAP.
  • This pattern is observed in various pulmonary pathologies, necessitating a differential diagnosis.

Purpose of the Study:

  • To provide a phenotype-first approach for differentiating PAP from other conditions with the crazy-paving pattern on HRCT.
  • To highlight key radiologic features, clinical context, and disease acuity for differential diagnosis.
  • To guide radiologists in recognizing PAP within the spectrum of airspace diseases.

Main Methods:

  • Review of representative imaging cases with a focus on the crazy-paving pattern.
  • Side-by-side comparison of imaging findings for PAP and its mimics.
  • Emphasis on radiologic distribution, clinical context, and disease acuity.

Main Results:

  • Distinguishing features between PAP and mimics like pulmonary edema, diffuse alveolar hemorrhage, organizing pneumonia, adenocarcinoma, lipoid pneumonia, and COVID-19 pneumonia are detailed.
  • Radiologic clues for autoimmune versus secondary PAP, including ground-glass opacities distribution, subpleural sparing, and lower lobe predominance, are identified.
  • Diagnostic pitfalls, especially post-pandemic, are discussed.

Conclusions:

  • A pattern-based framework aids radiologists in diagnosing PAP.
  • Understanding the differential diagnosis of the crazy-paving pattern is crucial for accurate patient management.
  • The review offers practical guidance for recognizing PAP and its mimics through imaging and clinical correlation.