Interstitial Lung Disease


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  • Interstitial Lung Disease Interstitial lung disease—sometimes called restrictive lung disease—refers to a group of lung problems. When you have interstitial lung disease, your lungs become inflamed and scarred. You may find it harder to take deep breaths. Or you may have a dry cough and mild chest discomfort. Interstitial lung disease is not asthma, although symptoms like shortness of breath may feel similar. Inside Your Lungs When you breathe, air travels in and out of your lungs through branching airways (bronchioles). Oxygen (O2) and carbon dioxide (CO2) are exchanged in the alveoli. Oxygen passes from the alveoli to the blood vessels through the tissue called interstitium.  The blood vessels then carry oxygen-rich blood to the rest of the body. Carbon dioxide moves back from the blood vessels to the alveoli and is then exhaled. How Lungs Become Damaged Interstitial lung disease develops in steps: First, the alveoli are injured and the lungs become inflamed. Then scarring of the lungs develops. The lungs may become stiff. With enough damage from scarring, oxygen can’t easily move through interstitium. Causes of Interstitial Lung Disease In most cases, interstitial lung disease has no known cause. Some known causes include: Dust from asbestos or silica, gases, fumes, or poisons Chemicals and drugs (chemotherapy or medications) Radiation therapy Lung infections Connective tissue disease (such as scleroderma, systemic lupus, or rheumatoid arthritis) Treatment for Interstitial Lung Disease Interstitial lung disease can’t be cured, but treatment can help you feel better. Treatment may include medication, breathing techniques, exercise, and stress management. In some cases, a lung transplant is an option. Your healthcare team may include: A primary care provider,  such as your family doctor. A pulmonologist,  a specialist in lung problems. A pulmonary nurse specialist  who helps you understand and carry out your treatment. A pulmonary rehabilitation specialist  who helps you gain strength through exercise. A social worker  who helps with your daily needs, family life, and stress.
  • Interstitial Lung Disease

    1. 1. Matthew Yambao Antonio Zuniga Katherine Phillips David Daliva
    2. 3. <ul><li>More than 140 different types of disease processes </li></ul><ul><li>many share a similar etiology </li></ul><ul><li>Pulmonary inflammation: common etiologic factor. </li></ul><ul><li>Similar pulmonary anatomic alterations </li></ul><ul><li>Abnormal tissue repair process: produces excess scar tissue </li></ul>
    3. 4. <ul><li>Cavity: a gas containing space surrounded by thick 1mm wall </li></ul><ul><li>Granuloma: small nodular mass of inflammatory cells (macrophages) which resemble epithelial cells </li></ul><ul><ul><ul><li>Granuloma formation: inflammatory response to infective and non infective agents </li></ul></ul></ul><ul><li>Honeycombing: numerous small air-containing cystic spaces with thickened walls composed of dense fibrous tissue </li></ul>
    4. 6. <ul><li>Inflammation & Edema secondary to: </li></ul><ul><li>Infiltration of WBCs (Leukocytes) </li></ul><ul><ul><ul><li>Into the alveolar walls and interstitial spaces </li></ul></ul></ul><ul><ul><ul><li>neutrophils, eosinophils, basophils, macrophages, monocytes, and lymphocytes </li></ul></ul></ul><ul><li>Sometimes: Bronchial inflammation and smooth muscle constriction (obstructive) </li></ul>
    5. 8. <ul><li>Increased numerous WBCs (including some fibroblasts) </li></ul><ul><li>Extensive inflammation: </li></ul><ul><ul><ul><li>Interstitium continues to thicken </li></ul></ul></ul><ul><ul><ul><li>Fibrosis and granulomas proliferate </li></ul></ul></ul><ul><ul><ul><li>Honeycombing and cavity formation ensue </li></ul></ul></ul><ul><ul><ul><li>Pleural effusions may occur </li></ul></ul></ul><ul><ul><ul><ul><ul><li>(end stage Pulmonary Fibrosis) </li></ul></ul></ul></ul></ul>
    6. 9. <ul><li>Fibrotic thickening of the bronchioles and alveolar units </li></ul><ul><li>Destruction of the alveoli and pulmonary capillaries </li></ul><ul><li>Honeycombing and cavity formation </li></ul><ul><li>Granulomas </li></ul><ul><li>Airway obstruction: secondary to inflammation and bronchial constriction </li></ul>
    7. 10. <ul><li>Inhalation of: pollen, animal dander, organic dusts, spores of molds </li></ul><ul><li>Farmers lung: moldy hay </li></ul><ul><li>Bird breeder's lung: bird droppings </li></ul><ul><li>Malt workers lung: moldy barley, malt dust </li></ul><ul><li>Maple bark pneumonitis: moldy Maple bark </li></ul><ul><li>Mushroom workers lung: mushroom compost </li></ul><ul><li>Wheat weevil disease: infested wheat flour </li></ul>
    8. 11. <ul><li>Inhalation of: pollen, animal dander, organic dusts, spores of molds </li></ul><ul><li>Farmers lung: moldy hay </li></ul><ul><li>Bird breeder's lung: bird droppings </li></ul><ul><li>Malt workers lung: moldy barley, malt dust </li></ul><ul><li>Maple bark pneumonitis: moldy Maple bark </li></ul><ul><li>Mushroom workers lung: mushroom compost </li></ul><ul><li>Wheat weevil disease: infested wheat flour </li></ul>
    9. 12. <ul><li>Inflammation and destruction of the pulmonary vessels </li></ul><ul><ul><ul><li>Wegener’s granulomatosis </li></ul></ul></ul><ul><ul><ul><li>Lymphomatoid granulomatosis </li></ul></ul></ul><ul><ul><ul><li>Chung-Strauss Syndrome </li></ul></ul></ul><ul><ul><ul><ul><ul><li>Rapidly fatal </li></ul></ul></ul></ul></ul><ul><ul><ul><li>Necrotizing vasculitis </li></ul></ul></ul>
    10. 13. <ul><li>Desquamative Interstitial Pneumonia (DIP) </li></ul><ul><ul><ul><li>Hyperplasia (excessive proliferation of tissue) </li></ul></ul></ul><ul><ul><ul><li>Desquamation (surface shedding) of alveolar type II cells </li></ul></ul></ul><ul><li>Usual Interstitial Pneumonia (UIP) </li></ul><ul><ul><ul><li>Thickening of the interstitium and alveolar walls </li></ul></ul></ul><ul><ul><ul><ul><ul><li>Severe cases: connective tissue replaces alveolar walls </li></ul></ul></ul></ul></ul><ul><li>Prognosis is better for DIP </li></ul>
    11. 14. <ul><li>Drug Induced ILD </li></ul><ul><ul><ul><li>Chemotherapeutical cytotoxic agents (anticancer agents) </li></ul></ul></ul><ul><ul><ul><ul><ul><li>Bleomycin, Cyclophosphamide </li></ul></ul></ul></ul></ul><ul><li>Radiation Induced ILD </li></ul><ul><ul><ul><li>almost always occurs with administration of doses above 6000 rads for more than 6 weeks </li></ul></ul></ul><ul><ul><ul><li>Fibrosis occurs 6-12 months after exposure </li></ul></ul></ul><ul><li>Irritant gas inhalation </li></ul>
    12. 15. <ul><li>Rheumatoid arthritis: when it involves the lungs </li></ul><ul><ul><ul><li>Atelectasis, pleurisy (with or without effusion) </li></ul></ul></ul><ul><li>Systemic lupus erythematosus (SLE) </li></ul><ul><ul><ul><li>Lungs , nervous system, heart </li></ul></ul></ul><ul><li>Progressive systemic sclerosis (scleroderma) </li></ul><ul><ul><ul><li>Lungs, pulmonary vasculature </li></ul></ul></ul><ul><li>Sjogrens Syndrome: salivary and lacrimal glands </li></ul><ul><ul><ul><li>Dry mucus membranes </li></ul></ul></ul><ul><ul><ul><li>Pleurisy, mucus plugging, atelectasis, secondary infections </li></ul></ul></ul>
    13. 16. <ul><li>Good Pastures Syndrome: affects the lungs and kidneys. </li></ul><ul><ul><ul><li>unknown etiology </li></ul></ul></ul><ul><ul><ul><li>pulmonary hemorrhage </li></ul></ul></ul><ul><ul><ul><li>increased DLCO: increased blood retained in the pulmonary tissue </li></ul></ul></ul><ul><li>Alveolar proteinosis: unknown etiology </li></ul><ul><ul><ul><li>alveoli fill with a lipoprotein material: similar to pulmonary surfactant </li></ul></ul></ul><ul><ul><ul><li>indistinguishable from pulmonary edema except for the absence of cardiac enlargement </li></ul></ul></ul>
    14. 17. <ul><li>Bronchiolitis obliterans with organizing pneumonia (BOOP) </li></ul><ul><ul><ul><li>plugs of connective tissue in the small airways (bronchiolitis obliterans) </li></ul></ul></ul><ul><ul><ul><li>infiltration of the surrounding parenchyma by mononuclear cells (organizing pneumonia) </li></ul></ul></ul><ul><li>Lymphangioleiomyomatosis (LAM) </li></ul><ul><ul><ul><li>rare disease: affects women of childbearing age </li></ul></ul></ul><ul><ul><ul><li>clinical complications: recurrent pneumothoraces, hemoptysis, and chylothorax </li></ul></ul></ul>
    15. 18. <ul><li>Hard metal dusts </li></ul><ul><ul><ul><li>cobalt and berrylium </li></ul></ul></ul><ul><li>Silica dust and asbestos fibers </li></ul><ul><li>Irritant gases </li></ul><ul><ul><ul><li>ammonia (commercial refrigeration, smelting of sulfide ores) </li></ul></ul></ul><ul><ul><ul><li>chlorine (chemical and plastic industries, water disinfection), </li></ul></ul></ul><ul><ul><ul><li>ozone (manufacture of bleaches and peroxides, welding) </li></ul></ul></ul>
    16. 19. <ul><li>Moldy hay, moldy barley, moldy sugarcane husks and mushroom compost </li></ul><ul><li>Airborne organic dusts or fibers </li></ul><ul><ul><ul><li>grain dust, dust from animal and bird droppings, cotton and hemp fibers, flax dust, malt dust and cork dust </li></ul></ul></ul><ul><li>Bacterial or fungal overgrowth may occur within air conditioners, air humidifiers, and hot tubs </li></ul><ul><li>Cigarette smoke should to be avoided </li></ul>
    17. 20. <ul><li>Excessive secretions not clinically manifested </li></ul><ul><li>Bronchopulmonary hygiene usually not indicated </li></ul><ul><li>General airway management </li></ul><ul><li>adequate humidification of inspired gas (if presence of a bypassed upper airway) </li></ul><ul><li>removal of secretions: endotracheal, tracheal, or nasotracheal suctioning as needed </li></ul><ul><li>Intubated Patient </li></ul><ul><li>management of cuff pressure using MLT or MOV technique </li></ul><ul><ul><ul><li>to avoid tracheal stenosis and/or tissue necrosis </li></ul></ul></ul><ul><li>Some cases of ILD may develop constriction of bronchial smooth muscle </li></ul><ul><ul><ul><li>aerosolized beta 2 adrenergic or anticholinergic medications can be admimistered to relieve the associated bronchospasm </li></ul></ul></ul>
    18. 21. SYMPTOMS OF ILD Symptoms of ILD SOB Dry cough Rapid, shallow breathing Chest discomfort Gradual, unintended weight loss Fatigue Aching muscles and joints Clubbing
    19. 22. SYMPTOMS MANAGEMENT Symptoms Management Avoid dust related irritants Avoid second hand smoke Be aware of one’s level of activity Breathing techniques Oxygen therapy Benefits of supplemental oxygen (Reduce heart strain, reduce SOB, alleviate chest discomfort, reduce fatigue, improve sleep) Devices that one’s doctor might order are: oxygen concentrator, liquid oxygen
    20. 23. BREATHING TRAINING TECHNIQUES Breathing Training Techniques Relax – reduces the amount of oxygen one’s body requires (conscious and deliberate effort is required) Diaphragmatic Breathing Pursed lip breathing
    21. 24. SMOKING CESSATION Ways to Quit Chantix Zyban Nicotine nasal spray Nicotine gum Nicotine patch Cold turkey
    22. 25. MEDICATIONS Medications <ul><li>Corticosteroids – anti-inflammatory drugs (Prednisone) </li></ul><ul><li>Cytotoxic Drugs – Azathioprine, Cyclophosphamide </li></ul><ul><li>  Antifibrotics </li></ul><ul><ul><li>Bosentan </li></ul></ul><ul><ul><li>Penicillamine </li></ul></ul><ul><li>Antioxidants </li></ul><ul><li>Oxygen </li></ul>
    23. 26. <ul><ul><ul><li>Why is it required as a part of pulmonary rehabilitation? </li></ul></ul></ul><ul><ul><ul><li>The benefits of having Relaxation Techniques </li></ul></ul></ul><ul><ul><ul><li>Ways to relax </li></ul></ul></ul>
    24. 27. <ul><ul><ul><li>Get into shape! </li></ul></ul></ul><ul><ul><ul><li>Benefits of Exercise </li></ul></ul></ul><ul><ul><ul><li>Safety Guidelines </li></ul></ul></ul><ul><ul><ul><ul><li>Know your limits </li></ul></ul></ul></ul>
    25. 28. <ul><ul><ul><li>What you should you eat? </li></ul></ul></ul><ul><ul><ul><li>Modified eating habits </li></ul></ul></ul><ul><ul><ul><li>Oxygen consumption of eating </li></ul></ul></ul><ul><ul><ul><li>Antioxidants </li></ul></ul></ul><ul><ul><ul><li>Losing Weight </li></ul></ul></ul>
    26. 29. <ul><ul><ul><li>Correct diagnosis </li></ul></ul></ul><ul><ul><ul><li>At home therapy </li></ul></ul></ul><ul><ul><ul><ul><li>Medicine </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Oxygen </li></ul></ul></ul></ul>
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