pulmonary emphysema

Introduction

Patients with pulmonary emphysema are the pink puffers of COPD.

Pathology

• centrilobular emphysema is most common type
  • generally begins in upper lobes
• panlobular emphysema is less common
  • generally begins in lower lobes
  • form of emphysema associated with alpha-1 antitrypsin deficiency
• lung compliance is increased
• elastic recoil is decreased
• cor pulmonale is rare

Clinical manifestations

• cough mild to moderate
• production of sputum is variable
• tachypnea
• purse-lipped breathing
• use of accessory muscles
• hyper-resonance to percussion
• decreased &/or adventitious breath sounds (wheezes, crackles, large airway sounds)
• symptoms tend to develop late since destruction of vascular supply generally accompanies airway destruction minimizing VQ mismatch
• infections may exacerbate symptoms
• dyspnea is moderate to marked
• severe weight loss may occur
• clubbing is rare

Laboratory

• alpha-1 antitrypsin in serum if family history

Diagnostic procedures

• pulmonary function testing
• pCO2 is normal is slightly increased
• decreased pO2
• DLCO is decreased
• FEV1 is decreased
• total lung volume is markedly increased
• residual volume is markedly increased

Radiology

• chest radiographs
  • often show flattened diaphragms
  • lung fields may be hyperlucent with diminished vascular markings
  • disease is often most prominent in upper lung fields
  • alpha-1 antitrypsin deficiency may show a basilar disease prominence & should be considered in patients < 50 years of age with emphysema
• computed tomography
  • significance of giant bullae
  • pre-op workup for lung reduction surgery
  • emphysema-like findings on CT associated with increased mortality, even in asymptomatic patients

Differential diagnosis

• alpha-1 antitrypsin deficiency
• cystic fibrosis
• ciliary dyskinesia
• bronchiectasis

Management

• see COPD
• lung volume reduction surgery
  • benefits patients with predominantly upper lobe emphysema
    • increased mortality when FEV1 < 20% of predicted
  • consider after course of pulmonary rehabilitation
  • appears to provide some benefit by improving mechanical function of thoracic cage compromised by hyperinflation of the lungs
  • endobronchial valve(s) may be of modest benefit at a cost of increased risk of pneumonia, COPD exacerbation
  • endobronchial coils
    • expensive, benefits are modest, not FDA approved
• lung transplantation

More general terms

• chronic obstructive pulmonary disease (COPD)
• emphysema

More specific terms

• compensatory emphysema
• giant bullous emphysema; Vanishing lung syndrome

Additional terms

• features of chronic bronchitis vs pulmonary emphysema

Patient information

pulmonary emphysema patient information