Interstitial Lung Disease MODULE G4 Chapter 28: pp

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.

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

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 PNEUMONOULTRAMICROSCOPICSILICOVOL CANOCONIOSIS

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

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

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

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

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

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

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

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

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

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

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

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