Interstitial Lung Disease

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Interstitial Lung Disease

• MODULE G4
• Chapter 28 pp. 379-396

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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.

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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

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Pneumoconiosis

• Asbestosis inhalation of asbestos fibers
• Coal Workers Pneumoconiosis coal dust
• Black lung or coal miners lung
• Silicosis inhalation of silica (rocks)
• Berylliosis inhalation of beryllium
• Siderosis inhalation of iron (welders)
• Talcosis inhalation of talcs
• PNEUMONOULTRAMICROSCOPICSILICOVOLCANOCONIOSIS

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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

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Hypersensitivity Pneumonitis

• Humidifier lung
• Bagassosis (sugar cane)
• Mushroom workers lung (mushroom compost)
• Farmers lung (hay)
• Grain handlers lung (grain)
• Bird Breeders lung (bird droppings feathers)
• Hot Tub Lung
• Sewer Workers Lung
• Fish Food Lung
• Sausage Workers Lung
• Woodworkers Lung
• Wine Growers Lung

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Drug Induced Lung Damage

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

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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

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Diseases of Unknown Etiology

• Idiopathic Pulmonary Fibrosis
• Sarcoidosis
• Goodpastures Syndrome

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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

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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

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Goodpastures Syndrome

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

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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

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Clinical Assessment

• Underlying Connective Tissue Disease
• Joint inflammation
• Joint deformities
• Muscle weakness
• Skin rash

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Pulmonary Functions

• Restrictive or Restrictive and Obstructive
• Decreased Lung Volumes and Flows
• Decreased DLCO
• Except for Goodpastures syndrome and
  hemosiderosis
• Smokers with IPF may have normal spirometry and
  lung volumes with a reduced DLCO (Emphysema
  IPF)

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Management

• Oxygen therapy
• Corticosteroids
• Sympathomimetics
• Cytotoxic Agents
• Vaccines (flu shots and pneumococcal)
• Plasmaphoresis Goodpastures syndrome
• Lung Transplantation
• Pulmonary Rehabilitation