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pulmonary emphysema

Patients with pulmonary emphysema are the pink puffers of COPD. Pathology: 1) centrilobular emphysema is most common type - generally begins in upper lobes 2) panlobular emphysema is less common a) generally begins in lower lobes b) form of emphysema associated with alpha-1 antitrypsin deficiency 3) lung compliance is increased 4) elastic recoil is decreased 5) cor pulmonale is rare Clinical manifestations: 1) cough mild to moderate 2) production of sputum is variable 3) tachypnea 4) purse-lipped breathing 5) use of accessory muscles 6) hyper-resonance to percussion 7) decreased &/or adventitious breath sounds (wheezes, crackles, large airway sounds) 8) symptoms tend to develop late since destruction of vascular supply generally accompanies airway destruction minimizing VQ mismatch 9) infections may exacerbate symptoms 10) dyspnea is moderate to marked 11) severe weight loss may occur 12) clubbing is rare [7] Laboratory: - alpha-1 antitrypsin in serum if family history Special laboratory: - pulmonary function testing 1) pCO2 is normal is slightly increased 2) decreased pO2 3) DLCO is decreased 4) FEV1 is decreased 5) total lung volume is markedly increased 6) residual volume is markedly increased Radiology: 1) chest radiographs a) often show flattened diaphragms b) lung fields may be hyperlucent with diminished vascular markings c) disease is often most prominent in upper lung fields d) alpha-1 antitrypsin deficiency may show a basilar disease prominence & should be considered in patients < 50 years of age with emphysema 2) computed tomography a) significance of giant bullae b) pre-op workup for lung reduction surgery c) emphysema-like findings on CT associated with increased mortality, even in asymptomatic patients [4] Differential diagnosis: 1) alpha-1 antitrypsin deficiency 2) cystic fibrosis 3) ciliary dyskinesia 4) bronchiectasis Management: 1) see COPD 2) lung volume reduction surgery a) benefits patients with predominantly upper lobe emphysema - increased mortality when FEV1 < 20% of predicted [6] b) consider after course of pulmonary rehabilitation c) appears to provide some benefit by improving mechanical function of thoracic cage compromised by hyperinflation of the lungs d) endobronchial valve(s) may be of modest benefit at a cost of increased risk of pneumonia, COPD exacerbation [3] e) endobronchial coils - expensive, benefits are modest, not FDA approved [5] 3) lung transplantation

Related

features of chronic bronchitis vs pulmonary emphysema

Specific

compensatory emphysema giant bullous emphysema; Vanishing lung syndrome

General

chronic obstructive pulmonary disease (COPD) emphysema

References

  1. Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 242
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 739
  3. Sciurba FC et al. A randomized study of endobronchial valves for advanced emphysema. N Engl J Med 2010 Sep 23; 363:1233 PMID: 20860505
  4. Oelsner EC et al Association Between Emphysema-like Lung on Cardiac Computed Tomography and Mortality in Persons Without Airflow Obstruction: A Cohort Study Ann Intern Med. 2014;161(12):863-873 PMID: 25506855 http://annals.org/article.aspx?articleid=2023010
  5. Deslee G et al. Lung volume reduction coil treatment vs usual care in patients with severe emphysema: The REVOLENS randomized clinical trial. JAMA 2016 Jan 12; 315:175. PMID: 26757466 - Sciurba FC et al. Bronchoscopic lung volume reduction in COPD: Lessons in implementing clinically based precision medicine. JAMA 2016 Jan 12; 315:139 PMID: 26757462
  6. Medical Knowledge Self Assessment Program (MKSAP) 17, American College of Physicians, Philadelphia 2015
  7. Geriatric Review Syllabus, 11th edition (GRS11) Harper GM, Lyons WL, Potter JF (eds) American Geriatrics Society, 2022