1. Interstitial Lung Disease

2. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 2 Organization of Interstitial Lung Disease (ILD)  Over 100 separate disorders under the auspices of ILD  Organized into subgroups of like disorders

3. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 3 Pathophysiology  Primarily a disease of the interstium  Repeated exposure to inflammatory agents or imperfect repair of damaged tissue leads to permanent damage.  Increased interstitial tissue replaces normal structures  Continuing injury or imperfect repair results in progressive damage and worsening impairment.  Physiological impairment due to damage  V/Q mismatch, shunt, ↓DLCO  Increased WoB due to decreased CL  These all lead to exercise intolerance...

4. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 4 Characteristics of ILD Clinical signs and symptoms of ILD Exertional dyspnea and nonproductive cough Exertional dyspnea and nonproductive cough  Most common reason to seek medical care May see increased: sputum production, hemoptysis, or wheezing May see increased: sputum production, hemoptysis, or wheezing Nonrespiratory symptoms may help identify presence of connective tissue disorder. Nonrespiratory symptoms may help identify presence of connective tissue disorder.

5. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 5 Characteristics of ILD Physical examination  On auscultation  Most commonly, bibasilar fine inspiratory crackles  In some disorders, will only hear diminished air entry i.e., sarcoidosis i.e., sarcoidosis  Wheezing is uncommon and probably due to a comorbidity.  Signs of right heart failure (late manifestation)  Pedal edema, JVD  May see features of underlying connective tissue disease

6. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 6 Characteristics of ILD (cont.) Chest radiographic features  Considerable variability dependent on specific disorder  Interstitial pulmonary fibrosis (IPF) has what is considered the classic ILD pattern.  Reduced volume  Bilateral, peripheral, basilar reticulonodular infiltrates  End-stage ILD presents with cystic honeycomb lung.  IPF is the second most common ILD (sarcoidosis first), and a number of other ILDs present in a similar manner.

7. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 7 Characteristics of ILD (cont.) Physiological features  Restrictive impairment is most common finding.  FEV 1 and FVC decreased while the FEV 1 /FVC ratio is normal to increased  Lung volumes and DLCO are reduced.  C L resulting in small V T and increased WOB  Less commonly, patients may have airflow obstruction.  May be sarcoidosis or some other mixed disease  Comorbidity with asthma or emphysema  May result in normal PFTs, but decreased DLCO

8. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 8 ILD: Exposure Related Asbestos-related pulmonary disease following exposure to asbestos is associated with  Pleural plaques, fibrosis, effusions, mesothelioma  Atelectasis, parenchymal scarring, lung cancer  Termed “asbestosis” if parenchymal fibrosis is present  Presents with slowly evolving DOE, inspiratory crackles  Shows typical PFTs, while chest radiograph often shows pleural change associated with asbestosis  Only supportive therapy is available.

9. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 9 ILD: Exposure Related (cont.) Chronic silicosis (inhaled silica particles)  Exposure: mining, sandblasting, and foundries  Chest radiograph shows apical nodular opacities  If these coalesce into large masses, it is called progressive massive fibrosis (PMF).  If impaired, patients often have a mixed obstructive and restrictive picture with a low DLCO.  Silicosis increases the odds of developing tuberculosis and lung cancer.

10. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 10 ILD: Exposure Related (cont.) Coal worker’s pneumoconiosis (CWP)  Used to think due to inhalation of silica, now understood that it is from a distinct exposure  Simple CWP asymptomatic, small nodules on radiograph  Cough and SoB if progresses to PMF similar to that seen in silicosis  No treatment for silicosis or CWP except stop exposure  Steroids and  2 -agonists for significant airway obstruction  Exacerbations treated with steroids and antibiotics

11. Chapter 25 Pneumoconiosis Chapter 25 Pneumoconiosis Figure 25-1. Pneumoconiosis, illustrated here in a case of asbestosis (close-up of one alveolar unit). AF, Asbestos fiber; FIB, fibrosis; M, macrophage. Inset, Cross-section showing fibrotic thickening of the alveolus, a common secondary anatomic alteration of the lungs.

12. Anatomic Alterations of the Lungs  Destruction of the alveoli and adjacent pulmonary capillaries  Fibrotic thickening of the respiratory bronchioles, alveolar ducts, and alveoli  Cystlike structures (honeycomb appearance)  Fibrocalcific pleural plaques (e.g., asbestosis)  Airway obstruction caused by inflammation and excessive bronchial secretions  Bronchospasm  Bronchogenic carcinoma  Mesothelioma (in asbestosis)

13. Etiology Etiologic Determinants  Size of inhaled particle  0.3 and 0.5 μm reach the alveoli  Chemical nature of the particle  Concentration of the particle  Length of exposure  The individual’s susceptibility

14. Etiology Asbestosis  Acoustic products  Automobile undercoating  Brake lining  Cements  Clutch casings  Floor tiles  Fire-fighting suits  Fireproof paints  Insulation  Roofing materials  Ropes  Steam pipe material

15. Etiology Coal Worker’s Pneumoconiosis  The deposition and accumulation of large amounts of coal dust cause what is know as coal worker’s pneumoconiosis (CWP)  Also called:  Coal miner’s lung  Black lung  Black phthisis  Miner’s phthisis

16. Etiology Silicosis  Tunneling  Hard-rock mining  Sandblasting  Quarrying  Stonecutting  Foundry work  Ceramics work  Abrasive work  Brick making  Paint making  Polishing  Stone drilling  Well drilling

17. Etiology Berylliosis  Beryllium is a steel-gray, lightweight metal found in:  Certain plastics and ceramics  Rocket fuels  X-ray

18. Etiology Other Forms of Pneumoconiosis  Aluminum  Ammunition workers  Baritosis (barium)  Barite millers and miners  Ceramics workers  Kaolinosis (clay)  Brick makers and potters  Ceramics workers  Siderosis (iron)  Welders  Talcosis (certain talcs)  Ceramics workers  Plastic and rubber workers

19. Overview of the Cardiopulmonary Clinical Manifestations Associated with PNEUMOCONIOSIS The following clinical manifestations result from the pathophysiologic mechanisms caused (or activated) by Increased Alveolar-Capillary membrane (see Figure 9-9), Bronchospasm (see Figure 9-10), and Excessive Bronchial Secretions (see Figure 9-11)—the major anatomic alterations of the lungs associated with chronic bronchitis (see Figure 25-1).

20. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 20 Medications, Drugs, and Radiation  Many drugs may cause ILD (see Box 24-1).  There is no specific pattern to drug-induced ILD  Diagnosis by known exposure to drug, subsequent development of ILD, and other causes are ruled out.  Treatment is avoidance of drug, possibly steroids  Cancer radiation therapy may result in ILD.  Presentation within 6 months with ground-glass appearance on chest radiograph, often responds to short-course steroids  Dyspnea presenting after 6 months, have dense fibrotic tissue, can only offer supportive therapy

21. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 21 Hypersensitivity Pneumonitis (HP)  A cell-mediated immune response to inhaled antigens  Patients must be sensitized by previous exposure.  Acute HP: patient presents with acute SoB, chest pain, fever, chills, malaise, and cough (may be productive)  Chronic HP: long-term antigen exposure leads to slow development over months to years  Presents with severe impairment that is difficult to distinguish from IPF

22. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 22 Hypersensitivity Pneumonitis (cont.)  Causative agents that can lead to HP  Common organic antigens (bacteria and fungi) found in Moldy hay (farmer’s lung) Moldy hay (farmer’s lung) Humidification systems (humidifier lung) Humidification systems (humidifier lung) Bird feces (bird breeders lung) Bird feces (bird breeders lung)  Inorganic antigens from paints and plastics  Treatment  Strict antigen identification and avoidance  Corticosteroids for symptomatic patients with acute HP Improves recovery but not lung function Improves recovery but not lung function  Chronic HP results in shorter survival and no known treatment

23. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 23 Systemic Disease Associated  ILD is a complication of various connective tissue diseases, most commonly  Scleroderma, rheumatoid arthritis, Sjögren’s syndrome, and systemic lupus erythematosus (SLE)  Significant pulmonary impairment prior to detection due to disease imposed sedentary lifestyle  Poor correlation between severity and pulmonary and nonpulmonary aspects of these diseases

24. Chapter 28 Chronic Interstitial Lung Diseases Figure 28-1. Chronic interstitial lung disease. Inset, Alveolar consolidation, a common secondary anatomic alteration of the lungs.

25. Anatomic Alterations of the Lungs  Fibrotic thickening of the respiratory bronchioles, alveolar ducts, and alveoli  Granulomas  Destruction of the alveoli and adjacent pulmonary capillaries  Honeycombing and cavity formation  Airway obstruction caused by inflammation and bronchial constriction

26. Etiology  More than 140 different disease processes are known to produce an interstitial lung disorder  No specific etiologic agent can be identified in more than 65% of the cases  In spite of the fact that a specific name may be attached to a particular disease entity  Box 28-1 lists some of the more common interstitial lung disorders

27. Box 28-1. Common Interstitial Lung Disorders.

28. Box 28-1. Common Interstitial Lung Disorders, cont.

30. Table 28-3.

31. Overview of the Cardiopulmonary Clinical Manifestations Associated with CHRONIC INTERSTITIAL LUNG DISEASES The following clinical manifestations result from the pathophysiologic mechanisms caused (or activated) by Increased Alveolar-Capillary Membrane Thickness (see Figure 9-9) and Bronchospasm (see Figure 9-10)—the major anatomic alterations of the lungs associated with chronic interstitial lung disease (see Figure 28-1)

32. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 32 Systemic Disease Associated (cont.)  Clinical manifestations vary  Dyspnea and cough are common.  Crackles, wheezes, and pleural rubs may be heard.  Typically restrictive disease  Depending on disease location, may be obstructive Sjögren’s disease in particular Sjögren’s disease in particular  ⇓ D LCO

33. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 33 Systemic Disease Associated (cont.)  HRCT results vary from normal to ground-glass appearance to noting reticular and fibrotic changes.  NSIP is associated with ground-glass appearance.  OP is associated with patchy consolidation with air bronchograms.  UIP is associated with reticular opacities, honeycomb  Treatment of systemic inflammatory diseases varies.  Most common treatment for acute inflammation or rapid progression is prolonged immunosuppression.  Typical agents: Cyclophosphamide, azathioprine Cyclophosphamide, azathioprine

34. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 34 Sarcoidosis  Most common ILD in United States.  Idiopathic multisystem inflammatory disease that forms granulomas in lungs but often follows benign course  Most common sign is asymptomatic hilar adenopathy.  If symptomatic: cough, chest pain, dyspnea, wheezing  Physiology may be normal, restrictive, obstructive, mixed  Steroids may be used for sickest symptomatic patients.

35. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 35 Interstitial Lung Diseases of Unknown Cause Idiopathic interstitial pneumonias (IIP)  Most common is IPF, a progressive fibrotic lung disorder  Mostly in older patients (>60 years of age)  Present with chronic cough and DOE  HRCT: bibasilar, peripheral reticular pattern, with cysts  Lung biopsy shows UIP and is diagnostic.  Most die within 4 years of progressive lung fibrosis.  There is no effective treatment. Oral steroids and azathioprine used but benefit few Oral steroids and azathioprine used but benefit few Otherwise, supportive therapy Otherwise, supportive therapy

36. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 36 Idiopathic Interstitial Pneumonias Nonspecific interstitial pneumonia (NSIP)  An IIP with diffuse inflammation on surgical lung biopsy most commonly tied to fibrosis forming fibrotic NSIP  On average occurs 7–10 years prior to IPF  Presents with chronic cough and dsypnea  HRCT shows ground glass (NSIP) or fibrotic changes and ground glass (fibrotic NSIP).  Prognosis is 7–10 years with immunosuppression (steroids and cytotoxic agents).

37. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 37 Idiopathic Interstitial Pneumonias (cont.) Cryptogenic organizing pneumonia (COP)  Patients are younger than those with IPF  A third have an antecedent viral illness.  Present with acute or subacute cough and dsypnea  HRCT findings typical for acute pneumonia, but patient failed to respond to several courses of antibiotics

38. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 38 Idiopathic Interstitial Pneumonias (cont.) Cryptogenic organizing pneumonia (COP) (cont.)  Most improve with course of oral steroids  Number relapse when steroids stopped  A few develop progressive fibrosis despite aggressive immunosuppression  Can offer lung transplantation

39. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 39 ILD With Distinct Pathology Lymphangioleiomyomatosis (LAM)  Rare, occurs mostly in women  Proliferation of smooth muscle around small airways leading to severe obstruction and destruction of alveoli  Leads to formation of thin walled cysts  Presents with DOE, obstructive impairment, ⇓ DLCO  Progression from hardly noticeable in older women to steadily progressive for middle-aged women

40. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 40 ILD With Distinct Pathology (cont.) Lymphangioleiomyomatosis (cont.)  Pleurodesis is generally required for recurrent chylothorax  Treatment:  Inhaled  2 -agonists and steroids  Younger patients may at some point receive lung transplantation

41. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 41 ILD With Distinct Pathology (cont.) Pulmonary Langerhans’ cell histiocytosis (PLCH)  ILD associated with adult smokers Present with DOE, cough, diffuse inspiratory crackles, airway obstruction, and ⇓ DLCO  HRCT: central mid-lung star-shaped nodules adjacent to thin-walled cysts  Primary treatment: Stop ALL exposure to tobacco smoke  If smoke avoidance fails to halt progression, can try Oral steroids: no proven benefit Oral steroids: no proven benefit  May offer lung transplantation

42. General Management of Chronic Interstitial Lung Diseases Respiratory care treatment protocols  Oxygen therapy protocol  Aerosolized medication protocol  Mechanical ventilation protocol

43. General Management of Chronic Interstitial Lung Diseases Medications and procedures commonly prescribed by the physician  Corticosteroids  Other agents  Cytotoxic agents are used to treat Wegener’s granulomatosis

44. General Management of Chronic Interstitial Lung Diseases Other treatments  Plasmapheresis  Used to treat Goodpasture’s syndrome Decreases the circulating anti-GBM antibodies Decreases the circulating anti-GBM antibodies

45. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 45 Nonspecific Interstitial Lung Disease Therapies  Oxygen therapy  Hypoxemia is common in ILD. Should be evaluated at rest and on exertion Should be evaluated at rest and on exertion  O 2 via nasal cannula may improve resting hypoxemia and allow greater exertion before desaturation May improve quality of life and avoid cor pulmonale May improve quality of life and avoid cor pulmonale  Pulmonary rehabilitation and exercise therapy  Not well studied  Encourage for all ILD patients to improve aerobic fitness, maintain activities, improve quality of life

46. Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. 46 Nonspecific Interstitial Lung Disease Therapies (cont.)  Vaccinations and infection avoidance  CDC recommends annual pneumococcal and influenza vaccine.  Patient should frequently wash hands.  May need pneumocystis prophylaxis if immunocompromised.  Transplantation  Only therapy shown to prolong life in end-stage ILD  Mortality is 10–25% at 1 year and 50–60% at 5 years.  Age (>65) and comorbidities often disqualify patients.