- •Table of Contents
- •Copyright
- •Dedication
- •Introduction to the eighth edition
- •Online contents
- •List of Illustrations
- •List of Tables
- •1. Pulmonary anatomy and physiology: The basics
- •Anatomy
- •Physiology
- •Abnormalities in gas exchange
- •Suggested readings
- •2. Presentation of the patient with pulmonary disease
- •Dyspnea
- •Cough
- •Hemoptysis
- •Chest pain
- •Suggested readings
- •3. Evaluation of the patient with pulmonary disease
- •Evaluation on a macroscopic level
- •Evaluation on a microscopic level
- •Assessment on a functional level
- •Suggested readings
- •4. Anatomic and physiologic aspects of airways
- •Structure
- •Function
- •Suggested readings
- •5. Asthma
- •Etiology and pathogenesis
- •Pathology
- •Pathophysiology
- •Clinical features
- •Diagnostic approach
- •Treatment
- •Suggested readings
- •6. Chronic obstructive pulmonary disease
- •Etiology and pathogenesis
- •Pathology
- •Pathophysiology
- •Clinical features
- •Diagnostic approach and assessment
- •Treatment
- •Suggested readings
- •7. Miscellaneous airway diseases
- •Bronchiectasis
- •Cystic fibrosis
- •Upper airway disease
- •Suggested readings
- •8. Anatomic and physiologic aspects of the pulmonary parenchyma
- •Anatomy
- •Physiology
- •Suggested readings
- •9. Overview of diffuse parenchymal lung diseases
- •Pathology
- •Pathogenesis
- •Pathophysiology
- •Clinical features
- •Diagnostic approach
- •Suggested readings
- •10. Diffuse parenchymal lung diseases associated with known etiologic agents
- •Diseases caused by inhaled inorganic dusts
- •Hypersensitivity pneumonitis
- •Drug-induced parenchymal lung disease
- •Radiation-induced lung disease
- •Suggested readings
- •11. Diffuse parenchymal lung diseases of unknown etiology
- •Idiopathic pulmonary fibrosis
- •Other idiopathic interstitial pneumonias
- •Pulmonary parenchymal involvement complicating systemic rheumatic disease
- •Sarcoidosis
- •Miscellaneous disorders involving the pulmonary parenchyma
- •Suggested readings
- •12. Anatomic and physiologic aspects of the pulmonary vasculature
- •Anatomy
- •Physiology
- •Suggested readings
- •13. Pulmonary embolism
- •Etiology and pathogenesis
- •Pathology
- •Pathophysiology
- •Clinical features
- •Diagnostic evaluation
- •Treatment
- •Suggested readings
- •14. Pulmonary hypertension
- •Pathogenesis
- •Pathology
- •Pathophysiology
- •Clinical features
- •Diagnostic features
- •Specific disorders associated with pulmonary hypertension
- •Suggested readings
- •15. Pleural disease
- •Anatomy
- •Physiology
- •Pleural effusion
- •Pneumothorax
- •Malignant mesothelioma
- •Suggested readings
- •16. Mediastinal disease
- •Anatomic features
- •Mediastinal masses
- •Pneumomediastinum
- •Suggested readings
- •17. Anatomic and physiologic aspects of neural, muscular, and chest wall interactions with the lungs
- •Respiratory control
- •Respiratory muscles
- •Suggested readings
- •18. Disorders of ventilatory control
- •Primary neurologic disease
- •Cheyne-stokes breathing
- •Control abnormalities secondary to lung disease
- •Sleep apnea syndrome
- •Suggested readings
- •19. Disorders of the respiratory pump
- •Neuromuscular disease affecting the muscles of respiration
- •Diaphragmatic disease
- •Disorders affecting the chest wall
- •Suggested readings
- •20. Lung cancer: Etiologic and pathologic aspects
- •Etiology and pathogenesis
- •Pathology
- •Suggested readings
- •21. Lung cancer: Clinical aspects
- •Clinical features
- •Diagnostic approach
- •Principles of therapy
- •Bronchial carcinoid tumors
- •Solitary pulmonary nodule
- •Suggested readings
- •22. Lung defense mechanisms
- •Physical or anatomic factors
- •Antimicrobial peptides
- •Phagocytic and inflammatory cells
- •Adaptive immune responses
- •Failure of respiratory defense mechanisms
- •Augmentation of respiratory defense mechanisms
- •Suggested readings
- •23. Pneumonia
- •Etiology and pathogenesis
- •Pathology
- •Pathophysiology
- •Clinical features and initial diagnosis
- •Therapeutic approach: General principles and antibiotic susceptibility
- •Initial management strategies based on clinical setting of pneumonia
- •Suggested readings
- •24. Bacterial and viral organisms causing pneumonia
- •Bacteria
- •Viruses
- •Intrathoracic complications of pneumonia
- •Respiratory infections associated with bioterrorism
- •Suggested readings
- •25. Tuberculosis and nontuberculous mycobacteria
- •Etiology and pathogenesis
- •Definitions
- •Pathology
- •Pathophysiology
- •Clinical manifestations
- •Diagnostic approach
- •Principles of therapy
- •Nontuberculous mycobacteria
- •Suggested readings
- •26. Miscellaneous infections caused by fungi, including Pneumocystis
- •Fungal infections
- •Pneumocystis infection
- •Suggested readings
- •27. Pulmonary complications in the immunocompromised host
- •Acquired immunodeficiency syndrome
- •Pulmonary complications in non–HIV immunocompromised patients
- •Suggested readings
- •28. Classification and pathophysiologic aspects of respiratory failure
- •Definition of respiratory failure
- •Classification of acute respiratory failure
- •Presentation of gas exchange failure
- •Pathogenesis of gas exchange abnormalities
- •Clinical and therapeutic aspects of hypercapnic/hypoxemic respiratory failure
- •Suggested readings
- •29. Acute respiratory distress syndrome
- •Physiology of fluid movement in alveolar interstitium
- •Etiology
- •Pathogenesis
- •Pathology
- •Pathophysiology
- •Clinical features
- •Diagnostic approach
- •Treatment
- •Suggested readings
- •30. Management of respiratory failure
- •Goals and principles underlying supportive therapy
- •Mechanical ventilation
- •Selected aspects of therapy for chronic respiratory failure
- •Suggested readings
- •Index
Upper airway disease
The obstructive diseases considered so far primarily affect the airways below the level of the main carina —the bronchi and bronchioles. In contrast to disease of these lower airways, a variety of other disorders affect the pharynx, larynx, and trachea and produce what is termed upper airway obstruction. The discussion of these disorders includes a brief consideration of representative etiologic factors and some of the tests used to make the diagnosis. In particular, use of the flow-volume loop to define the location of upper airway obstruction is considered.
Etiology
The upper airway can be affected by either acute problems or those following a more subacute or chronic course. On an acute basis, the larynx is probably the major area subject to obstruction. Potential causes include infection (epiglottitis, often due to H. influenzae), thermal injury and the resulting laryngeal edema from smoke inhalation, aspiration of a foreign body, laryngeal edema from an allergic (anaphylactic) reaction, or physical trauma associated with endotracheal intubation.
On a chronic basis, the upper airway may be partially obstructed by hypertrophy of the tonsils, by tumors (particularly of the trachea), by strictures of the trachea (often resulting from prior instrumentation of the trachea), or by vocal cord paralysis. Tracheomalacia, another chronic condition that may be congenital or acquired, is characterized by flaccidity of supporting airway cartilage and results in upper airway narrowing, especially on forced exhalation. The entity of obstructive sleep apnea, which is considered further in Chapter 18, is characterized by recurrent episodes of upper airway obstruction during sleep, resulting from anatomic factors and/or abnormal control of upper airway musculature.
Pathophysiology
The resistance of a tube to airflow varies inversely to the fourth power of the radius; hence, even small changes in airway size may produce dramatic changes in resistance and in the work of breathing. There are three pathophysiologic categories of upper airway obstruction which depend on the rigidity and site of the obstruction (intrathoracic vs. extrathoracic). A fixed obstruction occurs when the involved airway is rigidly narrowed, without any change in the size of the lumen during the respiratory cycle. In this case, inspiration and expiration are impaired by the same amount, and the flow rate generated during inspiration is essentially identical to the flow rate during expiration.
On the other hand, if airway diameter changes during the respiratory cycle, the greatest impairment to airflow occurs when the airway diameter is smallest. This type of obstruction is termed a variable obstruction. If the obstruction is located within the thorax, changes in pleural pressure during the respiratory cycle affect the size of the airway and therefore the magnitude of the obstruction. During a forced expiration, the positive pleural pressure causes airway narrowing, making the obstructing lesion more critical. In contrast, during inspiration, the airways increase their diameter, and the effects of a partial obstruction are less pronounced (see Fig. 3.22).
The location and respiratory variability of an upper airway obstruction affect the appearance of the flow-volume curve and the findings on physical examination.
In contrast, if the obstruction is located above the level of the thorax (i.e., outside the thorax), changes in pleural pressure are not directly transmitted to the airway in question. Rather, the negative airway pressure during inspiration tends to create a vacuum-like effect on extrathoracic upper airways, narrowing them and augmenting the effect of any partial obstruction. During expiration, the pressure
generated by the flow of air from the intrathoracic airways tends to widen the extrathoracic airways and decrease the net effect of a partially obstructing lesion (see Fig. 3.22).
Clinical features
Patients with upper airway obstruction may have dyspnea or cough. On physical examination, they may have evidence of flow through narrowed airways. If the lesion is variable and intrathoracic, the primary difficulty with airflow occurs during expiration, and patients demonstrate expiratory wheezing. If the lesion is variable and extrathoracic, obstruction is more marked during inspiration, and patients frequently manifest inspiratory stridor, a high-pitched, monophonic, continuous inspiratory sound often best heard over the trachea. With acute upper airway obstruction, such as that seen with inhalation of a foreign body, anxiety and respiratory distress often are apparent, signaling a medical emergency. In patients with epiglottitis, respiratory distress often is accompanied by sore throat, change in voice, dysphagia, and drooling.
Diagnostic approach
In the evaluation of suspected disorders of the upper airway, radiography and direct visualization provide the most useful information about the macroscopic appearance of the airway. Lateral neck radiographs or CT scans of the upper airway may reveal the localization, extent, and character of a partially obstructing lesion. A CT scan may offer particularly useful information by providing a cross-sectional view of the airways from the larynx down to the carina. Direct visualization of the upper airway may be obtained by laryngoscopy or bronchoscopy, which may reveal the presence of edema, vocal cord paralysis, or an obstructing lesion such as a tumor. However, direct visualization of the airways by these techniques includes some risk. The instrument used occupies part of the already compromised airway and may induce airway spasm or swelling that further obstructs the airway. This is especially true in cases of suspected epiglottitis, in which direct visualization should not be attempted unless the examiner is prepared to perform an emergency tracheostomy.
Because the functional consequences of a fixed versus a variable obstruction and an extrathoracic versus an intrathoracic obstruction are quite different, functional assessment of the patient with presumed upper airway obstruction can be useful in quantifying and localizing the obstruction. To recognize these distinctions, the flow-volume loop and the principles discussed in the pathophysiology section must be understood. This type of physiologic evaluation is appropriate for chronic upper airway obstruction, not for acute life-threatening obstruction.
When a fixed lesion is causing a relatively critical obstruction, maximal flow rates generated during inspiration and expiration are approximately equal, and a “plateau” marks both the inspiratory and expiratory parts of the flow-volume curve. When the lesion is variable, the effect of the obstruction depends on whether the lesion is intrathoracic or extrathoracic. With an intrathoracic obstruction, critical narrowing occurs during expiration, and the expiratory part of the flow-volume curve displays a plateau. With an extrathoracic obstruction, the expiratory part of the loop is preserved, and the inspiratory portion displays the plateau. A schematic diagram of the flow-volume loops observed in these types of upper airway obstruction is shown in Fig. 3.23.
Treatment
Because many different types of disorders result in upper airway obstruction, treatment varies greatly depending on the underlying problem, particularly its acuteness and severity. In acute severe upper airway obstruction, an emergency procedure such as endotracheal intubation or tracheostomy may be necessary to
maintain a patent airway. A procedure such as bronchoscopic laser therapy or airway stenting also may be
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used. Discussion of each disorder and further consideration of management can be found in other textbooks and in some articles listed in the Suggested Readings.
Suggested readings
Bronchiectasis
Boucher R.C. Muco-obstructive lung diseases New England Journal of Medicine 2019;380: 1941-1953.
Chalmers J.D, Chang A.B, Chotirmall S.H, Dhar R. & McShane P.J. Bronchiectasis Nature Reviews Disease Primers 2018;4: 45.
Chalmers J.D. & Chotirmall S.H. Bronchiectasis: New therapies and new perspectives
Lancet Respiratory Medicine 2018;6: 715-726.
Chang A.B, Fortescue R, Grimwood K, Alexopoulou E, Bell L, Boyd J., et al. European Respiratory Society guidelines for the management of children and adolescents with bronchiectasis European Respiratory Journal 2021;58: 2002990.
Gould C.M, Freeman A.F. & Olivier K.N. Genetic causes of bronchiectasis Clinics in Chest Medicine 2012;33: 249-263.
Hill A.T, Sullivan A.J, Chalmers J.D, De Soyza A, Elborn S.J, Floto A.R., et al. British Thoracic Society Guideline for bronchiectasis in adults Thorax Suppl. 1, 2019;74: 1-69.
Horani A. & Ferkol T.W. Advances in the genetics of primary ciliary dyskinesia: Clinical implications Chest 2018;154: 645-652.
Imam J.S. & Duarte A.G. Non-CF bronchiectasis: Orphan disease no longer Respiratory Medicine 2020;166: 105940.
Knowles M.R, Zariwala M. & Leigh M. Primary ciliary dyskinesia Clinics in Chest Medicine 2016;37: 449-461.
Lucas J.S, Davis S.D, Omran H. & Shoemark A. Primary ciliary dyskinesia in the genomics age Lancet Respiratory Medicine 2020;8: 202-216.
McShane P.J. & Tino G. Bronchiectasis Chest 2019;155: 825-833.
Nikolic A. Pathophysiology and genetics of bronchiectasis unrelated to cystic fibrosis Lung 2018;196: 383-392.
O’Connor M.G, Griffiths A, Iyer N.P, Shapiro A.J, Wilson K.C. & Thomson C.C. Summary for clinicians: Diagnosis of primary ciliary dyskinesia Annals of the American Thoracic Society 2019;16: 171-174.
O’Donnell A.E. Bronchiectasis-a clinical review New England Journal of Medicine 2022;387: 533-545.
Polverino E, Goeminne P.C, McDonnell M.J, Aliberti S, Marshall S.E, Loebinger M.R., et al.
European Respiratory Society guidelines for the management of adult bronchiectasis
European Respiratory Journal 2017;50: 1700629.
Verma N, Grimbacher B. & Hurst J.R. Lung disease in primary antibody deficiency Lancet Respiratory Medicine 2015;3: 651-660.
Cystic fibrosis
Cohen T.S. & Prince A. Cystic fibrosis: A mucosal immunodeficiency syndrome Nature Medicine 2012;18: 509-519.
De Boeck K. & Amaral M.D. Progress in therapies for cystic fibrosis Lancet Respiratory
Medicine 2016;4: 662-674.
Elborn J.S, Bell S.C, Madge S.L, Burgel P.R, Castellani C, Conway S., et al. Report of the European Respiratory Society/European Cystic Fibrosis Society task force on the care of adults with cystic fibrosis European Respiratory Journal 2016;47: 420-428.
Farrell P.M, White T.B, Ren C.L, Hempstead S.E, Accurso F, Derichs N., et al. Diagnosis of cystic fibrosis: Consensus guidelines from the Cystic Fibrosis Foundation Journal of Pediatrics 2017;181S: S4S15.e1.
Gentzsch M. & Mall M.A. Ion channel modulators in cystic fibrosis Chest 2018;154: 383393.
Heltshe S.L, Cogen J, Ramos K.J. & Goss C.H. Cystic fibrosis: The dawn of a new therapeutic era American Journal of Respiratory and Critical Care Medicine 2017;195: 979-984.
Jennings M.T. & Flume P.A. Cystic fibrosis: Translating molecular mechanisms into effective therapies Annals of the American Thoracic Society 2018;15: 897-902.
Mall M.A, Mayer-Hamblett N. & Rowe S.M. Cystic fibrosis: Emergence of highly effective targeted therapeutics and potential clinical implications American Journal of Respiratory and Critical Care Medicine 2020;201: 1193-1208.
Middleton P.G, Mall M.A, Drˇevínek P, Lands L.C, McKone E.F, Polineni D., et al.
Elexacaftor-tezacaftor-ivacaftor for cystic fibrosis with a single Phe508del allele New England Journal of Medicine 2019;381: 1809-1819.
Mingora C.M. & Flume P.A. Pulmonary complications in cystic fibrosis: Past, present, and future: Adult cystic fibrosis series Chest 2021;160: 1232-1240.
Rang C, Keating D, Wilson J. & Kotsimbos T. Re-imagining cystic fibrosis care: Next generation thinking European Respiratory Journal 2020;55: 1902443.
Stanford G.E, Dave K. & Simmonds N.J. Pulmonary exacerbations in adults with cystic fibrosis: A grown-up issue in a changing cystic fibrosis landscape Chest 2021;159: 93102.
Upper airway disease
Ernst A, Feller-Kopman D, Becker H.D. & Mehta A.C. Central airway obstruction American Journal of Respiratory and Critical Care Medicine 2004;169: 1278-1297.
Eskander A, de Almeida J.R. & Irish J.C. Acute upper airway obstruction New England Journal of Medicine 2019;381: 1940-1949.
Gaissert H.A. & Burns J. The compromised airway: Tumors, strictures, and tracheomalacia
Surgical Clinics of North America 2010;90: 1065-1089.
Grenier P.A, Beigelman-Aubry C. & Brillet P.Y. Nonneoplastic tracheal and bronchial stenoses Radiologic Clinics of North America 2009;47: 243-260.
Mudambi L, Miller R. & Eapen G.A. Malignant central airway obstruction Journal of Thoracic Disease Suppl. 10, 2017;9: S1087S1110.
Oberg C.L, Holden V.K. & Channick C.L. Benign central airway obstruction Seminars in Respiratory and Critical Care Medicine 2018;39: 731-746.
Petrov A.A. Vocal cord dysfunction: The spectrum across the ages Immunology and Allergy Clinics of North America 2019;39: 547-560.
Wright C.D. Tracheobronchomalacia and expiratory collapse of central airways Thoracic Surgery Clinics 2018;28: 163-166.
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