Ch23 Restrictive Pulmonary Disorders

  1. restrictive pulmonary disorders
    result from decreased lung expansion
  2. Restrictive pulmonary disorders 
    • decrease in vital capacity (VC), lung capacity (FRC), residual volume (RV)
    • greater the decrease in lung volume, greater the severity of disease
  3. Fibrotic interstitial lung disease 
    characterized by thickening of alveolar interstitium
  4. Diffuse Interstitial lung disease 
    • immune reaction
    • Inflammation 
    • Fibrosis
    • Destruction
  5. Diffuse Interstitial lung disease [pathogenesis]
    immune reaction
    • begins with injury to alveolar epithelial or capillary endothelial cells 
    • interstitial and alveolar wall thickening 
    • increased collagen bundles in interstitium 
    • lung tissue becomes infiltrated 
    • persistent alveolitis leads to obliteration of alveolar capillaries, reorganization of lung parenchyma, irreversible fibrosis 
    • lead to large air-filled sacs (cysts) with dilated terminal and respiratory bronchioles
  6. Diffuse Interstitial lung disease [pathogenesis]
    • occurs early, reversible 
    • triggering event leads to inflammatory response and increased inflammatory cells 
    • ---injury leads to increased membrane permeability and movement of fluid/debris into alveoli
  7. Diffuse Interstitial lung disease [pathogenesis]
    • fibroblastic proliferation and deposition of large amt of collagen 
    • caused by increased mesenchymal cells and fibroblasts in interstitium 
    • Alveolar walls become thickened w/ increased amts of fibrous tissue
  8. Diffuse Interstitial lung disease [pathogenesis]
    • end-stage disease 
    • loss of alveolar walls
  9. Diffuse Interstitial lung disease 
    [clinical manifestations]
    • progressive dyspnea w/ exercise w/ desaturation
    • rapid-shallow breathing 
    • irritating, nonproductive cough
    • clubbing of nail beds 
    • bibasilar end-expiratory crackles (Valcro rales) 
    • Cyanosis 
    • anorexia, wt loss 
    • inability to increase cardiac output with exercise
  10. Diffuse Interstitial lung disease
    • CXR
    • PFT 
    • open lung biopsy
    • transbronchial biopsy
    • Gallium 67 scan
    • bronchoalveolar lavage
  11. Diffuse Interstitial lung disease
    • smoking cessation 
    • avoid environmental exposure to cause 
    • anti-inflammatory agents 
    • immunosuppressive agents 
    • lung transplant
  12. Sarcoidosis 
    • chronic common in 3rd-4th decade of life 
    • Activation of alveolar macrophage to unknown trigger
  13. Sarcoidosis 
    development of multiple, uniform, noncaseating epithelioid granulomas
  14. Sarcoidosis 
    [clinical manifestations]
    • malaise, fatigue 
    • wt loss 
    • fever 
    • dyspnea of insidious onset 
    • dry, nonproductive cough
    • erythema nodosum 
    • macules, papules, hyperpigmentation, and subcutaneous nodules 
    • hepatosplenomegaly, lymphadenopathy
  15. Sarcoidosis 
    • leukopenia, anemia
    • increased eosinophil count, elevated sedimentation rate 
    • increased Ca levels 
    • elevated liver enzymes 
    • anergy 
    • elevated angiotensin-converting enzyme in active disease

    • Gallium 67 scan
    • PFTs
    • Bronchoalveolar lavage 
    • transbronchial lung biopsy
    • CXR
  16. Sarcoidosis 
    [stages 1]
  17. Sarcoidosis
    [stage 1]
    • good prognosis
    • hilar adenopathy alone
  18. Sarcoidosis
    Stage 2
    hilar adenopathy and bilateral pulmonary infiltrates
  19. Sarcoidosis 
    stage 3
    pulmonary infiltrates w/o adenopathy
  20. Sarcoidosis 
    stage 4
    • advanced fibrosis w/ evidence of honeycombing,
    • dilar retraction, bullae, cysts, and emphysema
  21. Sarcoidosis 
    • corticosteroids 
    • immunosuppressive agents
  22. Hypersensitivity pneumonitis 
    • aka extrinsic allergic alveolitis 
    • restrictive and occupational disease 
    • predominant in nonsmokers
  23. Hypersensitivity pneumonitis 
    • genetic predispose
    • agent suggest by pt history
    • agent confirmed by precipitating antibodies in serum
    • antigen combines with serum antibody in alveolar walls; leads to type III hypersensitivity rxn
    • antigen-antibody complexes then elicit granulomatous inflammation leading to lung tissue injury 
    • must have delayed hypersensitivity (type IV) rxn to antigen to develop pneumonitis
  24. hypersensitivity pneumonitis
    [clinical manifestations]
    • symptoms after 4-6 hrs and resolve in 18-24hrs 
    • general:chills, sweating, shivering 
    • myalgias 
    • nausea
    • malaise, lethargy 
    • headaches

    • Respiratory symptoms: 
    • dyspnea at rest 
    • dry cough
    • tachypnea
    • chest discomfort 

    • physical findings 
    • cyanosis
    • crackles in lung bases
  25. hypersensitivity pneumonitis 
    [clinical manifestations]
    • acute febrile episodes
    • progressive pulmonary fibrosis with cough
    • dyspnea, fatigue 
    • cor pulmonale (R sided heart failure due to lung disorders)
  26. hypersensitivity pneumonitis
    [clinical manifestations]
    Chronic (hallmark)
    progressive, diffuse pulmonary fibrosis in upper lobes
  27. hypersensitivity pneumonitis 
    • ID ofeending agent and prevent further exposure 
    • oral corticosteroids
  28. Occupational lung diseases
    • result from inhalation of toxic gases or foreign particles 
    • atmospheric pollutants have large effect on occupational respiratory diseases
  29. Occupational lung diseases 
    What particles
    • pneumoncniosis
    • anthracosis
    • silicosis
    • asbestosis
  30. occupational lung disease 
    [predisposing factors]
    • preexisting lung disease 
    • exposure to atmospheric pollutants 
    • duration of dust exposure 
    • amt of dust conc. 
    • particle size of pollutant
  31. Occupational lung disease 
    • pollutants interfere and paralyze cilia 
    • inference with ciliary action 
    • alveolar macrophages try to engulf and remove
    • macrophages secrete lysozymes to control foreign particles activity 
    • enzymes damage alveolar walls causing deposition of fibrous materials
  32. occupational lung disease 
    [clinical manifestations]
    • symptoms depend on predisposing factor 
    • pneumonconiosis produces no signs/symptoms in early stage 
    • usually symptom free up to 10-20 yrs with chronic exposure
  33. occupational lung disease 
    [late clinical features]
    • chronic hypoxemia
    • cor pulmonale 
    • respiratory failure
  34. Occupational lung disease 
    • preventive measures
    • corticosteroids 
    • bronchodilators 
    • O2 therapy
  35. ARDS - Acute respiratory distress syndrome 
    occurs in assoc with other pathophysio processes
  36. ARDS
    • severe trauma
    • sepsis
    • aspiration of gastric acid 
    • fat emboli syndrome 
    • shock
  37. ARDS 
    • widespread pulmonary inflammation leads to: 
    • noncardiogenic pulmonary edema assoc with leaky pulmonary capillaries 
    • atelectasis assoc. with lack of surfactant (decrease surface tension in small alveoli and prevents from collapsing) 
    • fibrosis(hyaline membranes)
  38. ARDS 
    [characteristic abnormalities]
    • injury to alveoli fr wide variety of disorders 
    • changes in alveolar diameter 
    • injury to pulmonary circulation 
    • disruptions in O2 transport and utilization
  39. ARDS
    common findings
    severe hypoxemia caused by intrapulmonary shunting of blood
  40. ARDS 
    [clinical manifestations]
    Early and Late
    • EArly: 
    • sudden marked respiratory distress
    • slight increase in pulse rate 
    • dyspnea
    • low PaO2
    • shallow, rapid breathing

    • Late: 
    • tachycardia 
    • tachypnea
    • hypotension
    • marked restlessness 
    • crackles, rhonchi on auscultation
    • use of accessory muscles 
    • intercostal and sternal retractions cyanosis
  41. ARDS 
    hypoxemia refractory to increased levels of supplemental O2
  42. ARDS 
    • mostly supportive 
    • ID underlying cause 
    • maintain fluid and electrolyte balance 
    • block system inflammatory cells
    • adequate oxygenation
  43. IRDS 
    infant respiratory distress syndrome 
    • Hyaline membrane disease 
    • syndrome of premature neonates 
    • primary cause: lack of surfactant 

    • risk factors: 
    • birth prior to 25 wks gestation 
    • birth at advanced gestational age 
    • poorly controleld diabetes in mother 
    • deliveries after antepartum hemorrhage
    • C section w/o antecedent labor 
    • perinatal asphyxia
    • 2nd twin
    • previous infant with RDS
    • Rh factor incomp.
  44. IRDS
    [clinical manifestations]
    • shallow respirations, diminished breath sounds 
    • intercostal, subcostal, sternal retractions 
    • flaring of nares 
    • hypotension, bradycardia
    • peripheral edema
    • low body temp 
    • oliguria
    • tachypnea
  45. IRDS 
    • prevent
    • maintain adequate oxygen level 
    • mechanical ventilation with PEEP or continuous positive airway pressure 
    • exogenous surfactant adm
    • high freq. ventilation
    • antibiotics 
    • supportive therapy
  46. Pneumothorax 
    • accumulation of air in the pleural space 
    • primary cause: also, trauma 
  47. pneumothorax 
    open 'sucking' chest wall wound 

    • air enters during inspiration but cannot escape during expiration 
    • --leads to shift of mediastinum 
  48. Pneumothorax 
    [clinical manifestations]
    • small pneumothoraces usually not detect on physical
    • tachycardia
    • decreased/abset breath sounds on affected side 
    • hyperresonance 
    • sudden chest pain on affected side 
    • dyspnea

    tension and large spontaneous pneumothorax = emergency situations 
  49. Pneumothorax 
    • management depends on severity of problem and cause of air leak 
    • lung collapse: may or may not hospitalized/ treat symptomatically 
    • --chest tube placement with H2O seal and suction 
  50. Pleural effusion 
    • collection of fluid/pus in pleural cavity as result of another disease process 
    • 5-15 ml of serous fluid contained in pleural space 
  51. Flail chest 
    • results from multiple rib fractures r/t trauma to chest wall 
    • ribs fractured at 2 distant sites 
  52. pneumonia 
    why and causes
    inflammatory rnx in alveoli and interstitium caused by infectious agent 

    • causes: 
    • aspiration of oropharyngeal secretions composed of normal bacterial flora/ gastric contents
    • inhalation of contaminants 
    • contamination fr. systemic circulation 
  53. Pneumonia
    • community acquired 
    • hospital acquired
    • bacterial (G(+)) - Staphyl & Strept
    • fungal (aspergillus) 
  54. Pneumonia 
    [clinical manifestations] 
    • severity of disease and patient age causes variation in symptoms 
    • crackles and bronchial breath sounds over affected lung tissue 
    • chills 
    • fever 
    • cough, purulent sputum 
  55. Pneumonia 
    antibiotic therapy 
  56. Pulmonary tuberculosis 
    • prior infection
    • malnourishment, immunosuppression
    • living overcrowded condition
    • incarcerated persons 
    • immigrants 
    • elderly 
  57. Pulmonary tuberculosis 
    [clinical manifestations]
    • history of contact with infected person 
    • low grade fever 
    • cough
    • night sweats 
    • fatigue 
    • wt loss 
    • malaise 
    • anorexia 
  58. Pulmonary tuberculosis
    • administer multiple drugs (antibiotics) which org is susceptible 
    • add at least 2 agents to drug regimen when tx failure is suspected 
Card Set
Ch23 Restrictive Pulmonary Disorders
restrictive pulmonary disorders