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Updated: Jun 28 2019

Restrictive Lung Disease Overview

  • Introduction
    • Definition
      • restricted lung expansion
      • results in reduced lung volumes (TLC and FVC)
    • Pathophysiology
      • poor breathing mechanics with normal lung function
        • muscle weakness
          • polio and myasthenia gravis
        • chest wall limitation
          • kyphosis, scoliosis, and morbid obesity
        • ↓ pleural compliance
          • mesothelioma
      • interstitial lung diseases (diffuse parenchymal lung disease - DLCO)
        • results in reduced lung compliance and increased elastic recoil
        • the primary mechanism of resting hypoxemia in patients with interstitial lung disease is a diffusion limitation, with DLCO defect contributing to hypoxemia with activity
        • increased expiratory flow rates due to increased radial traction (result of increased elastic recoil) on airway walls
          • prevents small degree of dynamic collapse of conducting airways found in normal lungs
        • types
          • acute
            • acute interstitial pneumonia (AIP)
              • presents similar to acute respiratory distress syndrome (ARDS)
            • neonatal respiratory distress syndrome
            • drug toxicity (bleomycin, busulfan, and amiodarone)
          • chronic
            • idiopathic interstitial pneumonias
              • most common is idopathic pulmonary fibrosis (IPF)
              • non-specific interstitial pneumonia, respiratory bronchiolitis, desquamative interstitial pneumonia, cryptogenic organizing pneumonia, and lymphocytic interstitial pneumonia
            • pneumoconioses
              • coal miner's
              • silicosis
              • asbestosis
            • sarcoidosis
              • may also show obstructive or mixed pattern on PFT's
            • pulmonary Langerhans cell histiocytosis
            • hypersensitivity pneumonitis (may be acute, subacute, or chronic)
            • lymphangiomyomatosis (primarily young women)
  • Studies
    • Pulmonary function tests
      • FEV1 sec/FVC ratio should be normal
        • approximately normal because both FEV1 sec and FVC are reduced
      • ↓ FVC and TLC
        • key finding for restrictive lung diseases (TLC must be reduced)
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