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Interstitial Lung Disease

Interstitial Lung Disease. MODULE G4 Chapter 28: pp. 379-396. Chronic Interstitial Lung Disease. A group of diseases that cause inflammation and fibrosis of the lower respiratory tract. Pulmonary Fibrosis Granulomas Destruction of alveoli and capillaries Honeycombing and cavity formation

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Interstitial Lung Disease

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  1. Interstitial Lung Disease MODULE G4 Chapter 28: pp. 379-396

  2. Chronic Interstitial Lung Disease • A group of diseases that cause inflammation and fibrosis of the lower respiratory tract. • Pulmonary Fibrosis • Granulomas • Destruction of alveoli and capillaries • Honeycombing and cavity formation • Airway obstruction • 140 different diseases and list is growing • Patients present with dyspnea and cough.

  3. Etiology • Pneumoconiosis • Dust/particulate matter inhalation from occupational or environmental exposure. • Chapter 26 • Hypersensitivity Pneumonitis • Drug Induced • Oxygen, Cancer drugs, Antibiotics • Connective Tissue (Collagen) Disease • Rheumatoid arthritis, Scleroderma, Lupus • Unknown Etiology - Idiopathic • Sarcoidosis, Idiopathic pulmonary fibrosis

  4. Pneumoconiosis • Asbestosis – inhalation of asbestos fibers • Coal Worker’s Pneumoconiosis – coal dust • Black lung or coal miner’s lung • Silicosis – inhalation of silica (rocks) • Berylliosis – inhalation of beryllium • Siderosis – inhalation of iron (welders) • Talcosis – inhalation of talcs • PNEUMONOULTRAMICROSCOPICSILICOVOLCANOCONIOSIS

  5. Hypersensitivity Pneumonitis • Extrinsic Allergic Alveolitis • Inhalation of organic antigens or chemicals • Allergic response by the body • 75% of cases are related to farming, birds, and water contamination. • Mold spores • Parasites • Animal/bird proteins • Thermophilic actinomycetes (bacteria) • Decomposition of vegetation & standing water

  6. Hypersensitivity Pneumonitis • Humidifier lung • Bagassosis (sugar cane) • Mushroom worker’s lung (mushroom compost) • Farmer’s lung (hay) • Grain handler’s lung (grain) • Bird Breeder’s lung (bird droppings & feathers) • Hot Tub Lung • Sewer Worker’s Lung • Fish Food Lung • Sausage Worker’s Lung • Woodworker’s Lung • Wine Grower’s Lung

  7. Drug Induced Lung Damage • Oxygen-Induced ARDS • Organizing Pneumonia • Amiodarone, Bleomycin, Tetracycline • Parenchymal Hemorrhage • Anticoagulants, Cocaine • Pleural Effusions • Amiodarone, Bleomycin, Methotrexate

  8. Connective Tissue Diseases • Rheumatoid Arthritis • Inflammation of the joints • Scleroderma • Skin and small blood vessels • Pulmonary involvement is most severe • Systemic Lupus • Joints, Skin, kidneys, lungs, CNS, Heart • Polymyositis • Inflammatory disorder of the striated muscles

  9. Diseases of Unknown Etiology • Idiopathic Pulmonary Fibrosis • Sarcoidosis • Goodpasture’s Syndrome

  10. Idiopathic Pulmonary Fibrosis • Hammon-Rich Syndrome • Males 40 – 70 years • Two major disease entities • Desquamative Interstitial Pneumonitis (DIP) • Usual interstitial Pneumonitis (UIP) • Diagnosis confirmed by open lung biopsy • Fatal in 4-10 years

  11. Sarcoidosis • Multi-system granulomatous disease • Lungs, lymph nodes, liver, spleen, skin, eyes, small bones of hands and feet, kidney, heart • 35-40 years of age • Mild to fatal symptoms • Enlarged lymph nodes if lung is involved

  12. Goodpasture’s Syndrome • Involved Lungs and Kidneys • Hemorrhage • Fibrosis • Circulating Antibodies (anti-GBM) • Seen in young adults • Survival period is 15 weeks from diagnosis • Increased DLCO

  13. Clinical Assessment • History is VERY important • Dyspnea on exertion/Dyspnea at rest • Low levels of oxygen – hypoxemia • Coughing – nonproductive • Clubbing • Cyanosis • Chest Pain • Pulmonary hypertension & Cor Pulmonale

  14. Clinical Assessment • Underlying Connective Tissue Disease • Joint inflammation • Joint deformities • Muscle weakness • Skin rash

  15. Pulmonary Functions • Restrictive or Restrictive and Obstructive • Decreased Lung Volumes and Flows • Decreased DLCO • Except for Goodpasture’s syndrome and hemosiderosis • Smokers with IPF may have normal spirometry and lung volumes with a reduced DLCO (Emphysema & IPF)

  16. Management • Oxygen therapy • Corticosteroids • Sympathomimetics • Cytotoxic Agents • Vaccines (flu shots and pneumococcal) • Plasmaphoresis – Goodpasture’s syndrome • Lung Transplantation • Pulmonary Rehabilitation

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