Interstitial Lung Disease

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Matthew Yambao Matthew Yambao Antonio Zuniga Antonio Zuniga Katherine Katherine Phillips Phillips David Daliva David Daliva

Transcript of Interstitial Lung Disease

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Matthew YambaoMatthew YambaoAntonio ZunigaAntonio ZunigaKatherine Katherine PhillipsPhillipsDavid DalivaDavid Daliva

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More than 140 different types of disease More than 140 different types of disease processes processes

many share a similar etiologymany share a similar etiology Pulmonary inflammation: common Pulmonary inflammation: common

etiologic factor. etiologic factor. Similar pulmonary anatomic alterationsSimilar pulmonary anatomic alterations Abnormal tissue repair process: produces Abnormal tissue repair process: produces

excess scar tissue excess scar tissue

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Cavity: a gas containing space surrounded Cavity: a gas containing space surrounded by thick 1mm wall by thick 1mm wall

Granuloma: small nodular mass of Granuloma: small nodular mass of inflammatory cells (macrophages) which inflammatory cells (macrophages) which resemble epithelial cells resemble epithelial cells

Granuloma formation: inflammatory Granuloma formation: inflammatory response to infective and non response to infective and non infective agents infective agents

Honeycombing: numerous small air-Honeycombing: numerous small air-containing cystic spaces with thickened containing cystic spaces with thickened walls composed of dense fibrous tissue walls composed of dense fibrous tissue

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Inflammation & Edema secondary to:Inflammation & Edema secondary to: Infiltration of WBCs (Leukocytes)Infiltration of WBCs (Leukocytes)

Into the alveolar walls and interstitial Into the alveolar walls and interstitial spacesspaces

neutrophils, eosinophils, basophils, neutrophils, eosinophils, basophils, macrophages, monocytes, and macrophages, monocytes, and lymphocyteslymphocytes

Sometimes:Sometimes: Bronchial inflammation and Bronchial inflammation and smooth muscle constriction (obstructive)smooth muscle constriction (obstructive)

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Increased numerous WBCs (including Increased numerous WBCs (including some fibroblasts)some fibroblasts)

Extensive inflammation:Extensive inflammation: Interstitium continues to thickenInterstitium continues to thicken Fibrosis and granulomas proliferateFibrosis and granulomas proliferate Honeycombing and cavity formation Honeycombing and cavity formation

ensueensue Pleural effusions may occurPleural effusions may occur

(end stage Pulmonary Fibrosis) (end stage Pulmonary Fibrosis)

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Fibrotic thickening of the bronchioles and Fibrotic thickening of the bronchioles and alveolar unitsalveolar units

Destruction of the alveoli and pulmonary Destruction of the alveoli and pulmonary capillariescapillaries

Honeycombing and cavity formationHoneycombing and cavity formation Granulomas Granulomas Airway obstruction: secondary to Airway obstruction: secondary to

inflammation and bronchial constrictioninflammation and bronchial constriction

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Inhalation of: Inhalation of: pollen, animal dander, pollen, animal dander, organic dusts, spores of moldsorganic dusts, spores of molds

Farmers lung: moldy hayFarmers lung: moldy hay Bird breeder's lung: bird droppingsBird breeder's lung: bird droppings Malt workers lung: moldy barley, malt dustMalt workers lung: moldy barley, malt dust Maple bark pneumonitis: moldy Maple Maple bark pneumonitis: moldy Maple

barkbark Mushroom workers lung: mushroom Mushroom workers lung: mushroom

compostcompost Wheat weevil disease: infested wheat flour Wheat weevil disease: infested wheat flour

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Inhalation of: Inhalation of: pollen, animal dander, pollen, animal dander, organic dusts, spores of moldsorganic dusts, spores of molds

Farmers lung: moldy hayFarmers lung: moldy hay Bird breeder's lung: bird droppingsBird breeder's lung: bird droppings Malt workers lung: moldy barley, malt dustMalt workers lung: moldy barley, malt dust Maple bark pneumonitis: moldy Maple Maple bark pneumonitis: moldy Maple

barkbark Mushroom workers lung: mushroom Mushroom workers lung: mushroom

compostcompost Wheat weevil disease: infested wheat flour Wheat weevil disease: infested wheat flour

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Inflammation and destruction of the Inflammation and destruction of the pulmonary vesselspulmonary vessels

Wegener’s granulomatosisWegener’s granulomatosis Lymphomatoid granulomatosis Lymphomatoid granulomatosis Chung-Strauss SyndromeChung-Strauss Syndrome

Rapidly fatalRapidly fatal

Necrotizing vasculitisNecrotizing vasculitis

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Desquamative Interstitial Pneumonia Desquamative Interstitial Pneumonia (DIP)(DIP)

Hyperplasia (excessive proliferation of Hyperplasia (excessive proliferation of tissue) tissue)

Desquamation (surface shedding) of Desquamation (surface shedding) of alveolar type II cells alveolar type II cells

Usual Interstitial Pneumonia (UIP)Usual Interstitial Pneumonia (UIP) Thickening of the interstitium and Thickening of the interstitium and

alveolar wallsalveolar walls Severe cases: connective tissue Severe cases: connective tissue

replaces alveolar wallsreplaces alveolar walls

Prognosis is better for DIPPrognosis is better for DIP

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Drug Induced ILDDrug Induced ILD Chemotherapeutical cytotoxic agents Chemotherapeutical cytotoxic agents

(anticancer agents)(anticancer agents) Bleomycin, CyclophosphamideBleomycin, Cyclophosphamide

Radiation Induced ILDRadiation Induced ILD almost always occurs with administration almost always occurs with administration

of doses above 6000 rads for more than of doses above 6000 rads for more than 6 weeks6 weeks

Fibrosis occurs 6-12 months after Fibrosis occurs 6-12 months after exposureexposure

Irritant gas inhalationIrritant gas inhalation

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Rheumatoid arthritis:Rheumatoid arthritis: when it involves the when it involves the lungslungs

Atelectasis, pleurisy (with or without Atelectasis, pleurisy (with or without effusion)effusion)

Systemic lupus erythematosus (SLE)Systemic lupus erythematosus (SLE) Lungs , nervous system, heartLungs , nervous system, heart

Progressive systemic sclerosis Progressive systemic sclerosis (scleroderma) (scleroderma)

Lungs, pulmonary vasculature Lungs, pulmonary vasculature Sjogrens Syndrome:Sjogrens Syndrome: salivary and lacrimal salivary and lacrimal

glands glands Dry mucus membranesDry mucus membranes Pleurisy, mucus plugging, atelectasis, Pleurisy, mucus plugging, atelectasis,

secondary infectionssecondary infections

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Good Pastures Syndrome:Good Pastures Syndrome: affects the affects the lungs and kidneys.lungs and kidneys.

unknown etiologyunknown etiology pulmonary hemorrhagepulmonary hemorrhage increased DLCO: increased blood increased DLCO: increased blood

retained in the pulmonary tissue retained in the pulmonary tissue Alveolar proteinosis:Alveolar proteinosis: unknown etiology unknown etiology

alveoli fill with a lipoprotein material: alveoli fill with a lipoprotein material: similar to pulmonary surfactantsimilar to pulmonary surfactant

indistinguishable from pulmonary edema indistinguishable from pulmonary edema except for the absence of cardiac except for the absence of cardiac enlargementenlargement

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Bronchiolitis obliterans with organizing Bronchiolitis obliterans with organizing pneumonia (BOOP) pneumonia (BOOP)

plugs of connective tissue in the small plugs of connective tissue in the small airways (bronchiolitis obliterans)airways (bronchiolitis obliterans)

infiltration of the surrounding parenchyma by infiltration of the surrounding parenchyma by mononuclear cells (organizing pneumonia)mononuclear cells (organizing pneumonia)

Lymphangioleiomyomatosis (LAM)Lymphangioleiomyomatosis (LAM) rare disease: affects women of childbearing rare disease: affects women of childbearing

ageage clinical complications: recurrent clinical complications: recurrent

pneumothoraces, hemoptysis, and pneumothoraces, hemoptysis, and chylothorax chylothorax

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Hard metal dustsHard metal dusts cobalt and berryliumcobalt and berrylium

Silica dust and asbestos fibersSilica dust and asbestos fibers Irritant gasesIrritant gases

ammonia (commercial refrigeration, ammonia (commercial refrigeration, smelting of sulfide ores)smelting of sulfide ores)

chlorine (chemical and plastic industries, chlorine (chemical and plastic industries, water disinfection),water disinfection),

ozone (manufacture of bleaches and ozone (manufacture of bleaches and peroxides, welding)peroxides, welding)

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Moldy hay, moldy barley, moldy sugarcane Moldy hay, moldy barley, moldy sugarcane husks and mushroom composthusks and mushroom compost

Airborne organic dusts or fibersAirborne organic dusts or fibers grain dust, dust from animal and bird grain dust, dust from animal and bird

droppings, cotton and hemp fibers, flax droppings, cotton and hemp fibers, flax dust, malt dust and cork dust dust, malt dust and cork dust

Bacterial or fungal overgrowth may occur Bacterial or fungal overgrowth may occur within air conditioners, air humidifiers, and within air conditioners, air humidifiers, and hot tubshot tubs

Cigarette smoke should to be avoided Cigarette smoke should to be avoided

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Excessive secretions not clinically manifestedExcessive secretions not clinically manifested Bronchopulmonary hygiene usually not indicated Bronchopulmonary hygiene usually not indicated General airway managementGeneral airway management adequate humidification of inspired gas (if presence adequate humidification of inspired gas (if presence

of a bypassed upper airway)of a bypassed upper airway) removal of secretions: endotracheal, tracheal, or removal of secretions: endotracheal, tracheal, or

nasotracheal suctioning as needednasotracheal suctioning as neededIntubated PatientIntubated Patient management of cuff pressure using MLT or MOV management of cuff pressure using MLT or MOV

techniquetechnique to avoid tracheal stenosis and/or tissue to avoid tracheal stenosis and/or tissue

necrosis necrosis Some cases of ILD may develop constriction of Some cases of ILD may develop constriction of

bronchial smooth muscle bronchial smooth muscle aerosolized beta 2 adrenergic or aerosolized beta 2 adrenergic or

anticholinergic medications can be anticholinergic medications can be admimistered to relieve the associated admimistered to relieve the associated bronchospasm bronchospasm

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SYMPTOMS OF ILDSYMPTOMS OF ILD

Symptoms of ILDSymptoms of ILD

SOBSOBDry coughDry coughRapid, shallow breathingRapid, shallow breathingChest discomfortChest discomfortGradual, unintended weight lossGradual, unintended weight lossFatigueFatigueAching muscles and jointsAching muscles and jointsClubbingClubbing

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SYMPTOMS MANAGEMENTSYMPTOMS MANAGEMENT

Symptoms ManagementSymptoms Management

Avoid dust related irritants Avoid dust related irritants Avoid second hand smoke Avoid second hand smoke Be aware of one’s level of activity Be aware of one’s level of activity Breathing techniques Breathing techniques Oxygen therapy Oxygen therapy Benefits of supplemental oxygen Benefits of supplemental oxygen (Reduce heart strain, reduce SOB, (Reduce heart strain, reduce SOB, alleviate chest discomfort, reduce alleviate chest discomfort, reduce fatigue, improve sleep)fatigue, improve sleep)Devices that one’s doctor might Devices that one’s doctor might order are: oxygen concentrator, order are: oxygen concentrator, liquid oxygen liquid oxygen

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BREATHING TRAINING TECHNIQUESBREATHING TRAINING TECHNIQUES

Breathing Training TechniquesBreathing Training Techniques

Relax – reduces the amount of oxygen Relax – reduces the amount of oxygen one’s body requires (conscious and one’s body requires (conscious and deliberate effort is required)deliberate effort is required)Diaphragmatic BreathingDiaphragmatic BreathingPursed lip breathingPursed lip breathing

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SMOKING CESSATIONSMOKING CESSATION

Ways to QuitWays to Quit

Chantix Chantix ZybanZybanNicotine nasal sprayNicotine nasal sprayNicotine gumNicotine gumNicotine patchNicotine patchCold turkeyCold turkey

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MEDICATIONSMEDICATIONS

MedicationsMedications

Corticosteroids – Corticosteroids – anti-inflammatory drugsanti-inflammatory drugs (Prednisone)(Prednisone)Cytotoxic Drugs – Azathioprine, Cytotoxic Drugs – Azathioprine, CyclophosphamideCyclophosphamide  AntifibroticsAntifibrotics

BosentanBosentanPenicillaminePenicillamine

Antioxidants Antioxidants OxygenOxygen

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• Why is it required as Why is it required as a part of pulmonary a part of pulmonary rehabilitation?rehabilitation?

• The benefits of The benefits of having Relaxation having Relaxation Techniques Techniques

• Ways to relaxWays to relax

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Get into shape! Get into shape! Benefits of ExerciseBenefits of Exercise Safety Guidelines Safety Guidelines

Know your limitsKnow your limits

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What you should you eat?What you should you eat? Modified eating habits Modified eating habits Oxygen consumption of eatingOxygen consumption of eating AntioxidantsAntioxidants Losing WeightLosing Weight

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Correct diagnosisCorrect diagnosis At home therapyAt home therapy

Medicine Medicine OxygenOxygen

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