Interstitial Lung Disease

MODULE G4Chapter 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• 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

• PNEUMONOULTRAMICROSCOPICSILICOVOLCANOCONIOSIS

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

• 35-40 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 DLCO

• Except for Goodpasture’s syndrome and hemosiderosis

• Smokers with IPF may have normal spirometry and lung volumes with a reduced DLCO (Emphysema & IPF)

Management• Oxygen therapy• Corticosteroids• Sympathomimetics• Cytotoxic Agents• Vaccines (flu shots and pneumococcal)• Plasmaphoresis – Goodpasture’s

syndrome• Lung Transplantation• Pulmonary Rehabilitation