Chronic p Ulm Deseases

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    CHRONICCHRONIC

    PULMONARY DISEASESPULMONARY DISEASES

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    CHRONIC OBSTRUCTIVECHRONIC OBSTRUCTIVE

    PULMONARY DISEASESPULMONARY DISEASES

    Chronic obstructive pulmonary diseaseChronic obstructive pulmonary disease

    (COPD) is a nonspecific term that describes(COPD) is a nonspecific term that describes

    patients with chronicpatients with chronic increased resistance toincreased resistance toconducting air & includes chronic obstructiveconducting air & includes chronic obstructive

    bronchitis, chronic obstructive emphysema,bronchitis, chronic obstructive emphysema,

    bronchiectasisbronchiectasis, chronic, chronic bronchiolitisbronchiolitis..

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    CHRONICCHRONIC

    PULMONARY DISEASESPULMONARY DISEASES

    Restrictive pulmonary diseases show evidenceRestrictive pulmonary diseases show evidence

    for a decrease in forced expiratory volume asfor a decrease in forced expiratory volume as

    measured bymeasured by spirometricspirometric pulmonary functionpulmonary functiontests.tests.

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    Chronic BronchitisChronic Bronchitis

    Chronic bronchitis is defined clinically as theChronic bronchitis is defined clinically as the

    presence of a chronic, productive coughpresence of a chronic, productive cough

    without a discernible cause for more than halfwithout a discernible cause for more than half

    of a 2of a 2--year period.year period. Chronic bronchitisChronic bronchitis isis

    primarily a disease of cigarette smokers. Inprimarily a disease of cigarette smokers. In

    fact, somefact, some 90%90% of all cases occur in smokers.of all cases occur in smokers.

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    Pathology:Pathology: Chronic bronchitis isChronic bronchitis is

    characterized by hyperplasia and hypertrophycharacterized by hyperplasia and hypertrophy

    of the mucusof the mucus--secreting cells and an increasedsecreting cells and an increased

    proportion of mucous to serous cells.proportion of mucous to serous cells.

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    Chronic bronchitis. The bronchial wall is thickened byChronic bronchitis. The bronchial wall is thickened byhypertrophy and hyperplasia of the mucushypertrophy and hyperplasia of the mucus--secreting glands.secreting glands.The Reid index is greater than 0.5. The submucosa showsThe Reid index is greater than 0.5. The submucosa showsincreased smooth muscle and mild chronic inflammation.increased smooth muscle and mild chronic inflammation.

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    Pathology:Pathology: Other morphologic changes are variableOther morphologic changes are variable

    and includeand include::

    (1)(1) excess mucus in the central and peripheral airways;excess mucus in the central and peripheral airways;

    (2)(2) thickening of the bronchial wall by mucous glandthickening of the bronchial wall by mucous gland

    enlargement and edema, which, in turn, leads toenlargement and edema, which, in turn, leads to

    encroachment on the bronchial lumen;encroachment on the bronchial lumen;(3)(3) an increase in goblet cells;an increase in goblet cells;

    (4)(4) increased amounts of smooth muscle, which mayincreased amounts of smooth muscle, which may

    indicate bronchial hyperreactivity.indicate bronchial hyperreactivity.

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    EmphysemaEmphysema

    Emphysema is enlargement of the airspacesEmphysema is enlargement of the airspaces

    distal to the terminal bronchioles, withdistal to the terminal bronchioles, with

    destruction of their walls but without fibrosis.destruction of their walls but without fibrosis.

    Emphysema is classified in anatomic terms,Emphysema is classified in anatomic terms,

    but the classification should not obscure thebut the classification should not obscure the

    fact that the severity of emphysema is morefact that the severity of emphysema is more

    important than the type.important than the type.

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    Pathogenesis:Pathogenesis:

    The major cause of emphysema is cigaretteThe major cause of emphysema is cigarette

    smoking, and moderate to severe emphysemasmoking, and moderate to severe emphysema

    is rare in nonsmokers. Increased numbers ofis rare in nonsmokers. Increased numbers of

    neutrophils, which contain serine elastase andneutrophils, which contain serine elastase and

    other proteases, are found in theother proteases, are found in the

    bronchoalveolar lavage fluid of smokers.bronchoalveolar lavage fluid of smokers.

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    Smoking also reduces theSmoking also reduces the aa11-- antitrypsinantitrypsin

    activity in the lung owing to oxidation ofactivity in the lung owing to oxidation ofmethionine residues in the enzyme. In thismethionine residues in the enzyme. In thisway, unopposed and increased elastolyticway, unopposed and increased elastolyticactivity leads to destruction of elastic tissue inactivity leads to destruction of elastic tissue in

    the walls of the distal airspaces, therebythe walls of the distal airspaces, therebyimpairing elastic recoil.impairing elastic recoil.

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    Pathology:Pathology: Emphysema is morphologicallyEmphysema is morphologically

    classified according to the location of theclassified according to the location of thelesions within the pulmonary acinuslesions within the pulmonary acinus

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    TypesTypes ofof emphysemaemphysema.. TheThe acinusacinus isis thethe unitunit gasgas--exchangingexchanging structurestructure ofofthethe lunglung distaldistal toto thethe terminalterminal bronchiolebronchiole.. ItIt consistsconsists of,of, inin order,order, respiratoryrespiratorybronchioles,bronchioles, alveolaralveolar ducts,ducts, alveolaralveolar sacs,sacs, andand alveolialveoli.. InIn centrilobularcentrilobular(proximal(proximal acinaracinar)) emphysema,emphysema, thethe respiratoryrespiratory bronchiolesbronchioles arearepredominantlypredominantly involvedinvolved.. InIn paraseptalparaseptal (distal(distal acinaracinar)) emphysema,emphysema, thethealveolaralveolar ductsducts areare particularlyparticularly affectedaffected.. InIn panacinarpanacinar ((panlobularpanlobular))emphysema,emphysema, thethe acinusacinus isis uniformlyuniformly damageddamaged..

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    Centrilobular Emphysema.Centrilobular Emphysema.This form ofThis form of

    emphysema is the most frequently encounteredemphysema is the most frequently encountered

    variant and the one that is usually associatedvariant and the one that is usually associated

    both with cigarette smoking and with clinicalboth with cigarette smoking and with clinical

    symptoms. Centrilobular emphysema issymptoms. Centrilobular emphysema is

    characterized by destruction of the cluster ofcharacterized by destruction of the cluster ofterminal bronchioles near the end of theterminal bronchioles near the end of the

    bronchiolar tree in the central pulmonarybronchiolar tree in the central pulmonary

    lobulelobule

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    CentrilobularCentrilobular emphysemaemphysema.. (A)(A) AA wholewhole mountmount ofof thethe leftleft lunglung ofof aa smokersmoker

    withwith mildmild emphysemaemphysema showsshows enlargedenlarged airair spacesspaces scatteredscattered throughoutthroughout bothboth

    lobes,lobes, whichwhich representrepresent destructiondestruction ofof thethe terminalterminal bronchiolesbronchioles inin thethe centralcentral

    partpart ofof thethe pulmonarypulmonary lobulelobule.. TheseThese abnormalabnormal spacesspaces areare surroundedsurrounded byby intactintact

    pulmonarypulmonary parenchymaparenchyma.. (B)(B) InIn aa moremore advancedadvanced casecase ofof centrilobularcentrilobular

    emphysema,emphysema, destructiondestruction ofof thethe lunglung hashas progressedprogressed toto produceproduce large,large, irregularirregular

    airair spacesspaces..

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    CentrilobularCentrilobular EmphysemaEmphysema.. TheThe enlargedenlarged

    respiratoryrespiratory bronchiolesbronchioles formform enlargedenlargedairspaces,airspaces, whichwhich areare separatedseparated fromfrom eacheachotherother andand fromfrom thethe lobularlobular septasepta bybynormalnormal alveolaralveolar ductsducts andand alveolialveoli.. AsAscentrilobularcentrilobular emphysemaemphysema progresses,progresses,thesethese distaldistal structuresstructures maymay alsoalso bebeinvolvedinvolved.. TheThe bronchiolesbronchioles proximalproximal toto thetheemphysematousemphysematous spacesspaces areare inflamedinflamed andandnarrowednarrowed.. CentrilobularCentrilobular emphysemaemphysema isismostmost severesevere inin thethe upperupper zoneszones ofof thethelung,lung, thethe upperupper lobe,lobe, andand thethe superiorsuperiorsegmentsegment ofof thethe lowerlower lobelobe..

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    Panacinar Emphysema.Panacinar Emphysema.In this type ofIn this type of

    emphysema, the acinus is uniformly involved,emphysema, the acinus is uniformly involved,with destruction of the alveolar septa from thewith destruction of the alveolar septa from the

    center to the periphery of the acinuscenter to the periphery of the acinus

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    PanacinarPanacinar emphysemaemphysema.. (A)(A) AA wholewhole mountmount ofof thethe leftleft lunglung fromfrom aa patientpatient withwith severesevere

    emphysemaemphysema revealsreveals widespreadwidespread destructiondestruction ofof thethe pulmonarypulmonary parenchyma,parenchyma, whichwhich inin

    somesome areasareas leavesleaves behindbehind onlyonly aa lacylacy networknetwork ofof supportingsupporting tissuetissue.. (B)(B) TheThe lunglung fromfrom

    thisthis pa-tientpa-tient withwith aa11--antitrypsinantitrypsin deficiencydeficiency showsshows aa panacinarpanacinar patternpattern ofof emphysemaemphysema..

    TheThe lossloss ofof alveolaralveolar wallswalls hashas resultedresulted inin markedlymarkedly enlargedenlarged airair spacesspaces..

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    InIn thethe finalfinal stage,stage, panacinarpanacinar emphysemaemphysema

    leavesleaves behindbehind aa lacylacy networknetwork ofof supportingsupportingtissuetissue ("cotton("cotton--candycandy lung")lung").. ThisThis variantvariant

    occursoccurs inin severalseveral differentdifferent situations,situations, butbut isis

    oftenoften foundfound inin cigarettecigarette smokerssmokers inin associationassociation

    withwith centrilobularcentrilobular emphysemaemphysema.. InIn suchsuch cases,cases,thethe panacinarpanacinar patternpattern tendstends toto occuroccur inin thethe

    lowerlower zoneszones ofof thethe lung,lung, whereaswhereas centrilobularcentrilobular

    emphysemaemphysema isis seenseen inin thethe upperupper zoneszones..

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    LocalizedLocalized EmphysemaEmphysema.. ThisThis condition,condition,

    whichwhich waswas previouslypreviously knownknown asas paraseptalparaseptal

    emphysema,emphysema, isis characterizedcharacterized byby thethe destructiondestruction

    ofof alveolialveoli andand resultingresulting emphysemaemphysema inin onlyonly

    oneone or,or, atat most,most, aa fewfew locations,locations, withwith thethe

    remainderremainder ofof thethe lungslungs beingbeing normalnormal..

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    Although it has no clinical significance itself,Although it has no clinical significance itself,

    rupture of an area of localized emphysemarupture of an area of localized emphysemaproduces spontaneous pneumothorax.produces spontaneous pneumothorax.

    Progression of localized emphysema can resultProgression of localized emphysema can result

    in a large area of destruction, termed ain a large area of destruction, termed a bulla.bulla.

    Bullae range in size from as small as 2 cm toBullae range in size from as small as 2 cm to

    large lesions that occupy an entire hemithorax.large lesions that occupy an entire hemithorax.

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    Both restrictive and obstructive lung diseases canBoth restrictive and obstructive lung diseases canaffect the pulmonary arterial circulation. The lossaffect the pulmonary arterial circulation. The lossof normal lung parenchyma leads to pulmonaryof normal lung parenchyma leads to pulmonaryhypertension that leads to thickening of the smallhypertension that leads to thickening of the smallarteries along with reduplication to form aarteries along with reduplication to form a

    plexiformplexiform lesion, as seen here in a peripherallesion, as seen here in a peripheralpulmonary artery.pulmonary artery.

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    Microscopically atMicroscopically at lowlow magnification, the loss ofmagnification, the loss of

    alveolar walls with emphysema is demonstrated.alveolar walls with emphysema is demonstrated.RemainingRemaining airspacesairspaces areare dilateddilated..

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    AtelectasisAtelectasis is the collapse of expanded lungis the collapse of expanded lung

    tissuetissue. If the supply of air is obstructed, the. If the supply of air is obstructed, theloss of gas from the alveoli to the blood leadsloss of gas from the alveoli to the blood leads

    to collapse of the af-fected region.to collapse of the af-fected region. AtelectasisAtelectasis

    is an important postoperative complication ofis an important postoperative complication of

    abdominal surgery, occurring because ofabdominal surgery, occurring because of

    mucous obstruction of a bronchus andmucous obstruction of a bronchus and

    diminished respiratory movement, which, indiminished respiratory movement, which, in

    turn, occurs because of postoperative pain.turn, occurs because of postoperative pain.

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    Atelectasis. The right lung of this infant is paleAtelectasis. The right lung of this infant is paleand expanded by air, whereas the left lung isand expanded by air, whereas the left lung is

    collapsed.collapsed.

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    Bronchiectasis is the irreversible dilatationBronchiectasis is the irreversible dilatation

    of bronchi as a consequence of destruction ofof bronchi as a consequence of destruction ofthe muscular and elastic elements of theirthe muscular and elastic elements of theirwalls.walls.

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    PathogenesisPathogenesis

    Bronchiectasis may result from mechanicalBronchiectasis may result from mechanical

    obstruction of central bronchi by inhaledobstruction of central bronchi by inhaled

    foreign bodies, tumors, mucous plugs inforeign bodies, tumors, mucous plugs in

    asthma, and compressive lymphadenopathy.asthma, and compressive lymphadenopathy.

    More commonly, it is not obstructive in originMore commonly, it is not obstructive in origin

    but, rather, a complication of respiratorybut, rather, a complication of respiratory

    infections or defects in the defenseinfections or defects in the defensemechanisms that protect the airways frommechanisms that protect the airways from

    infection.infection.

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    Localized, nonobstructiveLocalized, nonobstructive

    bronchiectasisbronchiectasis was once a commonwas once a commondisease, usually resulting fromdisease, usually resulting fromchildhood bronchopulmonarychildhood bronchopulmonaryinfections such as measles, pertussis,infections such as measles, pertussis,

    or other bacterial infections.or other bacterial infections.Although vaccines and antibioticsAlthough vaccines and antibioticshave reduced the frequency ofhave reduced the frequency of

    bronchiectasis, onebronchiectasis, one--half to twohalf to two--thirdsthirdsof all cases still follow aof all cases still follow abronchopulmonary infection.bronchopulmonary infection.

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    Generalized bronchiectasisGeneralized bronchiectasis (nonobstructive)(nonobstructive)

    is, I for the most part, secondary to inheritedis, I for the most part, secondary to inheritedimpairments I in host defense mechanisms orimpairments I in host defense mechanisms or

    acquired conditions that permit introduction ofacquired conditions that permit introduction of

    infectious organisms into the airways.infectious organisms into the airways.

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    PathologyPathology

    Generalized bronchiectasis is usually bilateralGeneralized bronchiectasis is usually bilateral

    and most common in the lower lobes.and most common in the lower lobes.

    Localized bronchiectasis may be situatedLocalized bronchiectasis may be situated

    wherever the obstruction or infection occurred.wherever the obstruction or infection occurred.

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    BronchiectasisBronchiectasis. The. The resectedresected upper lobe shows widely dilatedupper lobe shows widely dilatedbronchi, with thickening of the bronchial walls and collapsebronchi, with thickening of the bronchial walls and collapseand fibrosis of the pulmonary parenchymaand fibrosis of the pulmonary parenchyma

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    AsthmaAsthma

    AsthmaAsthma isis characterizedcharacterized byby variablevariable

    obstructionobstruction toto thethe flowflow ofof airair andand increasedincreased

    responsivenessresponsiveness ofof thethe airwaysairways toto aa varietyvariety ofof

    stimulistimuli.. ItIt isis characterizedcharacterized clinicallyclinically byby

    paroxysmsparoxysms ofof wheezing,wheezing, dyspnea,dyspnea, andand coughcough..

    WhenWhen severesevere acuteacute asthmaasthma isis unresponsiveunresponsive toto

    therapy,therapy, itit isis referredreferred toto asas statusstatus asthmaticusasthmaticus..

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    Pathogenesis:Pathogenesis: Asthma has been attributed toAsthma has been attributed to

    an increased airway responsiveness to anan increased airway responsiveness to aninflammatory reaction provoked by diverseinflammatory reaction provoked by diverse

    stimuli. The beststimuli. The best--studied situation associatedstudied situation associated

    with induction of asthma is the inhalation ofwith induction of asthma is the inhalation of

    allergens.allergens.

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    There are two major clinical forms of asthma thatThere are two major clinical forms of asthma that

    can overlap.can overlap.

    Extrinsic asthma: there is typically an associationExtrinsic asthma: there is typically an associationwithwith atopyatopy (allergies) mediated by type 1(allergies) mediated by type 1hypersensitivity, and asthmatic attacks arehypersensitivity, and asthmatic attacks areprecipitated by contact with inhaled allergens.precipitated by contact with inhaled allergens.This form occurs most often in childhood.This form occurs most often in childhood.

    Intrinsic asthma: asthmatic attacks areIntrinsic asthma: asthmatic attacks areprecipitated by respiratory infections, exposure toprecipitated by respiratory infections, exposure tocold, exercise, stress, inhaled irritants, and drugscold, exercise, stress, inhaled irritants, and drugs

    such as aspirin. Adults are most often affected.such as aspirin. Adults are most often affected.

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    PathogenesisPathogenesis ofof asthmaasthma.. ImmunologicallyImmunologically mediatedmediated asthmaasthma..AllergensAllergens interactinteract withwith immunoglobulinimmunoglobulin EE onon mastmast cells,cells, eithereitheronon thethe surfacesurface ofof thethe epitheliumepithelium or,or, whenwhen therethere isis abnormalabnormalpermeabilitypermeability ofof thethe epithelium,epithelium, inin thethe submucosasubmucosa.. MediatorsMediatorsareare releasedreleased andand maymay reactreact locallylocally oror byby reflexesreflexes mediatedmediatedthroughthrough thethe vagusvagus..

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    PathogenesisPathogenesis ofof asthmaasthma.. DischargeDischarge ofof eosinophiliceosinophilic granulesgranulesfurtherfurther impairsimpairs mucociliarymucociliary functionfunction andand damagesdamages thetheepithelialepithelial cellscells.. InIn turn,turn, epithelialepithelial cellcell injuryinjury stimulatesstimulates nervenerveendingsendings inin thethe mucosa,mucosa, therebythereby initiatinginitiating anan autonomicautonomicdischargedischarge thatthat contributescontributes toto airwayairway narrowingnarrowing andand mucusmucussecretionsecretion..

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    At high magnification, the numerousAt high magnification, the numerous eosinophilseosinophilsare prominent from their bright redare prominent from their bright red cytoplasmiccytoplasmicgranules in this case of bronchial asthma.granules in this case of bronchial asthma.

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    AnAn inhaledinhaled allergenallergen inin aa sensitizedsensitized personperson

    interactsinteracts withwith IgEIgE antibodyantibody thatthat isis boundbound toto thethe

    surfacesurface ofof mastmast cellscells interspersedinterspersed amongamong thethe

    epithelialepithelial cellscells ofof thethe bronchialbronchial mucosamucosa..

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    As a result, mast cells degranulate and releaseAs a result, mast cells degranulate and release

    mediators of type I (immediate) hypersensitivity,mediators of type I (immediate) hypersensitivity,

    including histamine, bradykinin,leukotrienes,including histamine, bradykinin,leukotrienes,

    prostaglandins,thromboxane A2, and plateletprostaglandins,thromboxane A2, and platelet--

    activating factor (PAF). These substances produce:activating factor (PAF). These substances produce:

    (1)(1) smooth muscle contraction,smooth muscle contraction,(2)(2) mucus secretion,andmucus secretion,and

    (3)(3) increased vascular permeability and edema, each ofincreased vascular permeability and edema, each of

    which is a potent, albeit reversible, cause of airwaywhich is a potent, albeit reversible, cause of airway

    obstruction.obstruction.

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    Allergic Asthma.Allergic Asthma.

    This is the most common form of asthma andThis is the most common form of asthma and

    is usually found in children. Commonis usually found in children. Commonallergens include pollens, animal hair or fur,allergens include pollens, animal hair or fur,

    and contamination of house dust with mites.and contamination of house dust with mites.

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    Infections.Infections.A common precipitating factor in childhoodA common precipitating factor in childhood

    asthma is a viral respiratory tract infectionasthma is a viral respiratory tract infection

    rather than allergic stimuli.rather than allergic stimuli.

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    ExerciseExercise--Induced Asthma.Induced Asthma.

    Exercise can precipitate some degree ofExercise can precipitate some degree ofbronchospasm in the majority (65%) of allbronchospasm in the majority (65%) of allpatients with asthma, and in some patients,patients with asthma, and in some patients,exercise may be the only inciting factor. Theexercise may be the only inciting factor. The

    more rapid the ventilation (severity ofmore rapid the ventilation (severity ofexercise) and the colder and drier the air that isexercise) and the colder and drier the air that isbreathed, the more likely it is that an attack ofbreathed, the more likely it is that an attack ofasthma will occur.asthma will occur.

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    Occupational Asthma.Occupational Asthma.

    More than 80 different occupational exposuresMore than 80 different occupational exposures

    have been linked to the development ofhave been linked to the development of

    asthma. In some instances, these substancesasthma. In some instances, these substances

    provoke allergic asthma byprovoke allergic asthma by IgEIgE--relatedrelated

    hypersensitivity mechanisms. Those affectedhypersensitivity mechanisms. Those affectedinclude animal handlers, bakers, and workersinclude animal handlers, bakers, and workers

    exposed to wood and vegetable dusts, metalexposed to wood and vegetable dusts, metal

    salts, pharmaceutical agents, and industrialsalts, pharmaceutical agents, and industrial

    chemicals.chemicals.

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    DrugDrug--Induced Asthma.Induced Asthma.

    DrugDrug--induced bronchospasm occurs mostinduced bronchospasm occurs most

    commonly in patients with known asthma. Thecommonly in patients with known asthma. The

    best known of these compounds is aspirin, butbest known of these compounds is aspirin, but

    nonsteroidal antinonsteroidal anti--inflammatory agents haveinflammatory agents have

    also been implicated.also been implicated.

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    Air Pollution.Air Pollution.

    Massive air pollution, usually in associationMassive air pollution, usually in association

    with temperature inversions, is associated withwith temperature inversions, is associated with

    bronchospasm in patients with asthma andbronchospasm in patients with asthma and

    other preexisting lung conditions.other preexisting lung conditions.

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    Emotional Factors. Psychological stress canEmotional Factors. Psychological stress can

    aggravate or precipitate an attack ofaggravate or precipitate an attack ofbronchospasm in as many as half of all patientsbronchospasm in as many as half of all patients

    with asthma.with asthma.

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    These lungs appear essentially normal, but are normalThese lungs appear essentially normal, but are normal--appearing because they are theappearing because they are the hyperinflatedhyperinflated lungs of alungs of apatient who died with statuspatient who died with status asthmaticusasthmaticus..

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    Pathology: Most information regarding thePathology: Most information regarding the

    pathology of asthma has been derived frompathology of asthma has been derived fromautopsies of patients who died in statusautopsies of patients who died in status

    asthmaticus; thus, the most severe lesions areasthmaticus; thus, the most severe lesions are

    described. On gross examination, the lungs aredescribed. On gross examination, the lungs areremarkably distended with air, and he airwaysremarkably distended with air, and he airways

    are filled with thick, tenacious, and adherentare filled with thick, tenacious, and adherent

    mucous plugs.mucous plugs.

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    Pathology: Microscopically, the plugs containPathology: Microscopically, the plugs contain

    strips of epithelium and many eosinophils,strips of epithelium and many eosinophils,the extruded granules of which coalesce tothe extruded granules of which coalesce to

    form needleform needle--like crystals (Charcotlike crystals (Charcot--LeydenLeyden

    crystals). In some cases, the mucoid exudatecrystals). In some cases, the mucoid exudateforms a cast of the airwaysforms a cast of the airways

    (Curschmann spirals), which may be expelled(Curschmann spirals), which may be expelled

    with coughing.with coughing.

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    Asthma. A section of lung from a patient who diedAsthma. A section of lung from a patient who diedin status asthmaticus reveals a bronchus containing ain status asthmaticus reveals a bronchus containing aluminal mucous plug, submucosal gland hyperplasia,luminal mucous plug, submucosal gland hyperplasia,and smooth muscle hyperplasia.and smooth muscle hyperplasia.

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    Asthma. Higher magnification shows hyaline thickening ofAsthma. Higher magnification shows hyaline thickening ofthe subepithelial basement membrane and markedthe subepithelial basement membrane and markedinflammation of the bronchiolar wall, with numerousinflammation of the bronchiolar wall, with numerouseosinophils. The mucosa exhibits an inflamed and metaplasticeosinophils. The mucosa exhibits an inflamed and metaplasticepithelium.epithelium.

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    Between the bronchial cartilage at the right and theBetween the bronchial cartilage at the right and thebronchial lumen filled with mucus at the left is abronchial lumen filled with mucus at the left is a submucosasubmucosawidened by smooth muscle hypertrophy, edema, andwidened by smooth muscle hypertrophy, edema, andinflammation (mainlyinflammation (mainly eosinophilseosinophils). These are changes of). These are changes ofbronchial asthma. The peripheralbronchial asthma. The peripheral eosinophileosinophil count or thecount or thesputumsputum eosinophilseosinophils can be increased during an asthmaticcan be increased during an asthmatic

    attack.attack.

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    This cast of the bronchial tree is formed ofThis cast of the bronchial tree is formed of inspissatedinspissated

    mucus and was coughed up by a patient during anmucus and was coughed up by a patient during anasthmatic attack. The outpouring of mucus fromasthmatic attack. The outpouring of mucus fromhypertrophied bronchialhypertrophied bronchial submucosalsubmucosal glands, theglands, thebronchoconstrictionbronchoconstriction, and dehydration all contribute to the, and dehydration all contribute to theformation of mucus plugs that can block airways information of mucus plugs that can block airways inasthmatic patients.asthmatic patients.

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    Pathology: The epithelium displays a loss ofPathology: The epithelium displays a loss of

    the normal, pseudostratified appearance andthe normal, pseudostratified appearance andmay be denuded, with only the basal cellsmay be denuded, with only the basal cells

    remaining. The basal cells are hyperplastic,remaining. The basal cells are hyperplastic,

    and squamous metaplasia is seen. An increaseand squamous metaplasia is seen. An increase

    in the number of goblet cells is also apparent.in the number of goblet cells is also apparent.

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    Pathology: Characteristically, the epithelialPathology: Characteristically, the epithelial

    basement membrane is thickened owing to anbasement membrane is thickened owing to anincrease in collagen deep to the true basalincrease in collagen deep to the true basallamina. One of the most characteristic featureslamina. One of the most characteristic featuresof status asthmaticus is the prominence ofof status asthmaticus is the prominence of

    bronchial smooth muscle, which reflectsbronchial smooth muscle, which reflectsmuscle hyperplasia. The submucosa ismuscle hyperplasia. The submucosa isedematous and contains a mixed inflammatoryedematous and contains a mixed inflammatoryinfiltrate, including variable numbers ofinfiltrate, including variable numbers of

    eosinophils.eosinophils.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    A large number of pulmonary disordersA large number of pulmonary disordersare grouped as interstitial, infiltrative,are grouped as interstitial, infiltrative,or restrictive diseases, because theyor restrictive diseases, because theyare characterized by inflammatoryare characterized by inflammatory

    infiltrates in the interstitial space andinfiltrates in the interstitial space andhave similar clinical and radiologichave similar clinical and radiologicpresentations.presentations.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    Hypersensitivity PneumonitisHypersensitivity PneumonitisHypersensitivity pneumonitis refers toHypersensitivity pneumonitis refers toa group of immunologically mediateda group of immunologically mediatedconditions caused by exposure toconditions caused by exposure toorganic dusts, in which the alveoli andorganic dusts, in which the alveoli anddistal airways are preferentiallydistal airways are preferentiallyinvolved.involved.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    Hypersensitivity PneumonitisHypersensitivity PneumonitisPathogenesis:Pathogenesis: More than 30More than 30environmental antigens are known toenvironmental antigens are known toproduce hypersensitivity pneumonitis.produce hypersensitivity pneumonitis.Inhalation of these antigens leads toInhalation of these antigens leads toacute oracute or chronic interstitialchronic interstitialinflammation in the lung.inflammation in the lung.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    HypersensitivityHypersensitivity PneumonitisPneumonitis

    PathogenesisPathogenesisMost of the responsible antigens areMost of the responsible antigens areencountered in occupational settings, andencountered in occupational settings, and

    the diseases are often labeled according tothe diseases are often labeled according tothe specific occupation. For example,the specific occupation. For example,farmers exposed to moldy hay suffer fromfarmers exposed to moldy hay suffer fromfarmer's lung.farmer's lung. Sugar cane workers exposedSugar cane workers exposedto moldy, pressed sugarto moldy, pressed sugar cane (cane (bagassebagasse))

    acquireacquire bagassosisbagassosis; and bird breeders who; and bird breeders whocome in contact with feathers, serum, andcome in contact with feathers, serum, andexcrement of pigeons have pigeon breeder'sexcrement of pigeons have pigeon breeder'sdisease.disease.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    Hypersensitivity PneumonitisHypersensitivity PneumonitisPathogenesisPathogenesis

    Hypersensitivity pneumonitisHypersensitivity pneumonitisrepresents a combination of immunerepresents a combination of immunecomplexcomplex--mediated (type III) and cellmediated (type III) and cell--mediated (type IV) hypersensitivitymediated (type IV) hypersensitivityreactions, although the precisereactions, although the precise

    contribution of each is still beingcontribution of each is still beingdebated.debated.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    Hypersensitivity PneumonitisHypersensitivity PneumonitisPathogenesisPathogenesis

    Whereas acute hypersensitivityWhereas acute hypersensitivitypneumonitis is characterized by apneumonitis is characterized by aneutrophilic infiltrate in the alveoli andneutrophilic infiltrate in the alveoli andrespiratory bronchioles, the morerespiratory bronchioles, the morechronic lesions display mononuclearchronic lesions display mononuclear

    cells and granulomas, which arecells and granulomas, which aretypical of delayed hypersensitivity.typical of delayed hypersensitivity.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    HypersensitivityHypersensitivity PneumonitisPneumonitisPathology:Pathology: In the acute phase ofIn the acute phase ofhypersensitivityhypersensitivitypneumonitispneumonitis, bronchiolar necrosis, an, bronchiolar necrosis, an

    eosinophiliceosinophilic infiltrate,infiltrate, vasculitisvasculitis, and, andinterstitial pneumonia are present.interstitial pneumonia are present.Chronic disease is characterized byChronic disease is characterized by

    extensive interstitialextensive interstitial pneumonitispneumonitis,,with a dense infiltrate ofwith a dense infiltrate oflymphocytes and a few plasma cells inlymphocytes and a few plasma cells inthe alveolarthe alveolar walls.walls.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    HypersensitivityHypersensitivity PneumonitisPneumonitis

    PathologyPathologyMild, diffuse alveolar damage is usuallyMild, diffuse alveolar damage is usuallypresent,present,with hyperplasia of type IIwith hyperplasia of type II pneumocytespneumocytes..

    There is alsoThere is also a significant bronchiolar infiltrate,a significant bronchiolar infiltrate,sometimes withsometimes with bronchiolitisbronchiolitis obliteransobliterans. Most. Mostcharacteristic is the presence of scattered,characteristic is the presence of scattered,poorly formedpoorly formed granulomasgranulomas that contain foreignthat contain foreignbody giant cells. In the chronic end stage, thebody giant cells. In the chronic end stage, theinterstitial inflammation recedes. However,interstitial inflammation recedes. However,fibrosis is more apparent, the lung architecturefibrosis is more apparent, the lung architectureis distorted, and honeycombing occursis distorted, and honeycombing occurs..

    INTERSTITIAL LUNG DISEASESINTERSTITIAL LUNG DISEASES

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    Hypersensitivity PneumonitisHypersensitivity Pneumonitis

    PathologyPathology

    Hypersensitivity pneumonitis. A lung biopsy shows amild peribronchiolar chronic inflammatory interstitialinfiltrate, with a focus of intraluminal organizing

    fibrosis.

    INTERSTITIAL LUNG DISEASESINTERSTITIAL LUNG DISEASES

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    Hypersensitivity PneumonitisHypersensitivity Pneumonitis

    PathologyPathology

    Focal poorly formedgranulomas werescattered in the lungbiopsy.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    Hypersensitivity PneumonitisHypersensitivity PneumonitisClinical Features:Clinical Features: HypersensitivityHypersensitivitypneumonitispneumonitismay present as acute, subacute, ormay present as acute, subacute, or

    chronic pulmonarychronic pulmonary disease, dependingdisease, dependingon the frequency and intensity ofon the frequency and intensity ofexposure to the offending antigen. Theexposure to the offending antigen. The

    prototype of hypersensitivityprototype of hypersensitivitypneumonitis is farmer's lung, which ispneumonitis is farmer's lung, which iscaused by inhalation of thermophiliccaused by inhalation of thermophilicactinomycetes that grow in moldy hay.actinomycetes that grow in moldy hay.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    Hypersensitivity PneumonitisHypersensitivity PneumonitisClinical FeaturesClinical Features

    Typically, a farm worker enters a barnTypically, a farm worker enters a barnwhere hay has been stored for winterwhere hay has been stored for winterfeeding. After a lag period of severalfeeding. After a lag period of severalhours, the worker rapidly developshours, the worker rapidly developsdyspnea, cough, and mild fever. Thedyspnea, cough, and mild fever. The

    symptoms remit within 24 to 48 hourssymptoms remit within 24 to 48 hoursbut return on reexposure and, withbut return on reexposure and, withtime, become chronic.time, become chronic.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    Hypersensitivity PneumonitisHypersensitivity PneumonitisClinical FeaturesClinical Features

    Pulmonary function studies show aPulmonary function studies show arestrictive pattern, which isrestrictive pattern, which ischaracterized by decreasedcharacterized by decreasedcompliance, reduced diffusioncompliance, reduced diffusioncapacity, and hypoxemia. In thecapacity, and hypoxemia. In the

    chronic stage of hypersensitivitychronic stage of hypersensitivitypneumonitis, airway obstruction maypneumonitis, airway obstruction maybecome troublesome.become troublesome.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    SarcoidosisSarcoidosis

    SarcoidosisSarcoidosis is a chronic disease ofis a chronic disease ofunknown cause in whichunknown cause in which noncaseatingnoncaseatinggranulomasgranulomas occur in almost any organoccur in almost any organof the body.of the body. The lung is mostThe lung is mostfrequently involved, but the lymphfrequently involved, but the lymph

    nodes, skin, and eye are also commonnodes, skin, and eye are also commontargets.targets.

    INTERSTITIAL LUNG DISEASESINTERSTITIAL LUNG DISEASES

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    SarcoidosisSarcoidosis

    Organs commonly affected by sarcoidosis.Sarcoidosis involves many organs, most commonly

    the lymph nodes and lung.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    SarcoidosisSarcoidosis

    Epidemiology:Epidemiology: Sarcoidosis is aSarcoidosis is aworldwide disease affecting all racesworldwide disease affecting all racesand both sexes. In North America,and both sexes. In North America,

    sarcoidosis occurs much moresarcoidosis occurs much morefrequently in blacks than in whites,frequently in blacks than in whites,with the ratio being approximatelywith the ratio being approximately

    15:1.15:1.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    SarcoidosisSarcoidosis

    EpidemiologyEpidemiologyThe disease is often encountered in theThe disease is often encountered in theScandinavian countries, where theScandinavian countries, where theprevalence is 64 per 100,000prevalence is 64 per 100,000persons(compared with 10 per 100,000 inpersons(compared with 10 per 100,000 inFrance and 3 per 100,000 in Poland). It hasFrance and 3 per 100,000 in Poland). It hasbeen reported that the prevalence ofbeen reported that the prevalence ofsarcoidosissarcoidosis in Irishin Irish.. Nonspecific polyclonalNonspecific polyclonal

    activation ofB cells by Tactivation ofB cells by T--helper cells leadshelper cells leadstoto hyperglobulinemiahyperglobulinemia, which is, which is characteristiccharacteristicof activeof active sarcoidosissarcoidosis..

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    SarcoidosisSarcoidosis

    Pathogenesis:Pathogenesis: There is a consensusThere is a consensusthat sarcoidosis represents anthat sarcoidosis represents anexaggerated cellular immune responseexaggerated cellular immune response

    on the part of helper/inducer Ton the part of helper/inducer Tlymphocytes to unknown exogenouslymphocytes to unknown exogenousantigens or autoantigens. These cellsantigens or autoantigens. These cells

    accumulate in the affected organs,accumulate in the affected organs,where they secrete lymphokines andwhere they secrete lymphokines andrecruit macrophages that participate inrecruit macrophages that participate information of noncaseating granulomasformation of noncaseating granulomas..

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    SarcoidosisSarcoidosis

    Pathology:Pathology: Pulmonary sarcoidosisPulmonary sarcoidosismost commonly affects the lung andmost commonly affects the lung andhilar lymph nodes. Histologically,hilar lymph nodes. Histologically,

    multiple sarcoid granulomas aremultiple sarcoid granulomas arescattered in the interstitium of thescattered in the interstitium of thelung. The central part of thelung. The central part of the

    granuloma may be fibrotic andgranuloma may be fibrotic andsurrounded by palisaded histiocytes.surrounded by palisaded histiocytes.

    INTERSTITIAL LUNG DISEASESINTERSTITIAL LUNG DISEASES

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    SarcoidosisSarcoidosis

    PathologyPathology

    Sarcoidosis. Multiple noncaseating granulomasare present along the bronchovascularinterstitium

    INTERSTITIAL LUNG DISEASESINTERSTITIAL LUNG DISEASES

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    SarcoidosisSarcoidosis

    PathologyPathology

    Sarcoidosis. Noncaseating granulomas consist oftight clusters of epithelioid macrophages andmultinucleated giant cells.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    Usual Interstitial PneumoniaUsual Interstitial Pneumonia

    Usual interstitial pneumonia (UIP)Usual interstitial pneumonia (UIP)is the most common type of idiopathicis the most common type of idiopathicinterstitial pneumonitis and isinterstitial pneumonitis and is

    characterized clinically by progressivecharacterized clinically by progressiverespiratory insufficiency andrespiratory insufficiency andpathologically by interstitialpathologically by interstitial

    inflammation and fibrosis.inflammation and fibrosis. The diseaseThe diseaseaffects persons of all ages, with aaffects persons of all ages, with amean age at onset of 50 to 60 years.mean age at onset of 50 to 60 years.

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    Usual Interstitial PneumoniaUsual Interstitial Pneumonia

    Pathogenesis:Pathogenesis: It is generally held that UIPIt is generally held that UIPhas an immunologic basis. Approximatelyhas an immunologic basis. Approximately20% of the cases are associated with20% of the cases are associated with

    collagen vascular diseases, includingcollagen vascular diseases, includingrheumatoid arthritis, systemic lupusrheumatoid arthritis, systemic lupuserythematosuserythematosus, and progressive systemic, and progressive systemicsclerosis. The disease also occurs in thesclerosis. The disease also occurs in the

    context of other autoimmune disorders,context of other autoimmune disorders,such as Hashimotosuch as Hashimoto thyroiditisthyroiditis, primary, primarybiliarybiliary cirrhosis, idiopathic thrombocytopeniccirrhosis, idiopathic thrombocytopenicpurpurapurpura, and myasthenia gravis., and myasthenia gravis.

    INTERSTITIAL LUNG DISEASESINTERSTITIAL LUNG DISEASES

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    Usual Interstitial PneumoniaUsual Interstitial Pneumonia

    PathologyPathology

    Usual interstitial pneumonitis. A gross specimen ofthe lung shows patchy dense scarring withextensive areas of honeycomb cystic change.

    INTERSTITIAL LUNG DISEASESINTERSTITIAL LUNG DISEASES

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    INTERSTITIALLUNG DISEASESINTERSTITIALLUNG DISEASES

    Usual Interstitial PneumoniaUsual Interstitial Pneumonia

    PathologyPathology

    Usual interstitial pneumonitis. A microscopic viewdiscloses patchy interstitial dense fibrosis andinterstitial chronic inflammation. The areas ofdense fibrosis display remodeling, with loss of the

    normal lun architecture.

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    PULMONARY NEOPLASMSPULMONARY NEOPLASMS

    Lung tumours may be primary orLung tumours may be primary orsecondary. Both are commonsecondary. Both are common

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    PULMONARY NEOPLASMSPULMONARY NEOPLASMS

    Primary carcinoma of the lungPrimary carcinoma of the lung

    Most common primary malignantMost common primary malignanttumour in the worldtumour in the world

    Directly related to cigarette smokingDirectly related to cigarette smoking Associated with occupationalAssociated with occupational

    exposure to carcinogensexposure to carcinogens

    Overall 5Overall 5--year survival rate of 4year survival rate of 4--7%7% Squamous cell, smallSquamous cell, small

    cell,adenocarcinoma, and large cellcell,adenocarcinoma, and large cellundifferentiated typesundifferentiated types

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    PULMONARY NEOPLASMSPULMONARY NEOPLASMS

    Over 90% of primary lung tumoursOver 90% of primary lung tumoursare carcinomas. Lung cancer is theare carcinomas. Lung cancer is theleading cause of death from cancerleading cause of death from cancer

    in the world, with the worst overallin the world, with the worst overallprognosis, typically around 5% 5prognosis, typically around 5% 5--year survival.This is due to theyear survival.This is due to the

    aggressive natural history of theaggressive natural history of thedisease,only about 10% of casesdisease,only about 10% of casesbeing operable at diagnosis.being operable at diagnosis.

    SMALL CELL CARCINOMASMALL CELL CARCINOMA

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    SMALL CELL CARCINOMASMALL CELL CARCINOMA

    squamoussquamous cell carcinomacell carcinoma

    epidemiology and risk factors:epidemiology and risk factors:cigarette smoke; more common incigarette smoke; more common inmen; occurs on areas ofmen; occurs on areas of squamoussquamous

    metaplasiametaplasia (due to irritants)(due to irritants)

    macroscopic features:macroscopic features: typicallytypicallycentral and close to carinacentral and close to carina

    (frequently presenting with bronchial(frequently presenting with bronchialobstruction)obstruction)

    Carcinoma of the lungCarcinoma of the lung

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    Carcinoma of the lungCarcinoma of the lung

    SquamousSquamous Cell CarcinomaCell Carcinoma

    PathologyPathology:: MostMost squamoussquamous cellcellcarcinomas arise in the central portioncarcinomas arise in the central portionof the lung, from the major orof the lung, from the major or

    segmental bronchi. On grosssegmental bronchi. On grossexamination, they tend to be firm,examination, they tend to be firm,graygray--white, and ulcerated lesions thatwhite, and ulcerated lesions that

    extend through the bronchial wall intoextend through the bronchial wall intothe adjacent parenchyma.the adjacent parenchyma.

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    clinical presentation:clinical presentation: patientpatient

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    clinical presentation:clinical presentation: patientpatient

    may have no signsmay have no signs signs the same as those in metastaticsigns the same as those in metastatic

    tumourstumours::

    coughcough (80% of cases) due to infection distal to(80% of cases) due to infection distal toairway blocked byairway blocked by tumourtumour

    haemoptysishaemoptysis (70% of cases) due to ulceration of(70% of cases) due to ulceration oftumourtumour in bronchusin bronchus

    dyspnoeadyspnoea (60% of cases) due to local extension(60% of cases) due to local extensionofof tumourtumour

    chest painchest pain (40% of cases) due to involvement of(40% of cases) due to involvement of

    pleura and chest wallpleura and chest wall wheezewheeze (15% of cases) due to narrowing of(15% of cases) due to narrowing of

    airwayairway

    nonnon--specific systemic signs:specific systemic signs: weight loss,weight loss,anorexia, malaiseanorexia, malaise

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    stagingstagingtumourtumour

    stagingstaging tumourtumour -- gives angives anindication of severity and prognosisindication of severity and prognosis

    stages are classified from 1 (very goodstages are classified from 1 (very good

    prognosis) to 4 (very bad prognosis)prognosis) to 4 (very bad prognosis) staging is based on:staging is based on:

    topography (size, direct extension,topography (size, direct extension,obstruction)obstruction)

    nodal stasisnodal stasis

    metastasismetastasis

    Carcinoma of the lungCarcinoma of the lung

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    Carcinoma of the lungCarcinoma of the lung

    SquamousSquamous Cell CarcinomaCell Carcinoma

    PathologyPathology

    Carcinomas of the lung, of allCarcinomas of the lung, of all histologichistologictypes, metastasize most frequentlytypes, metastasize most frequently to theto the

    regional lymph nodes, particularly theregional lymph nodes, particularly the hilarhilarandand mediastinalmediastinal nodes. The most commonnodes. The most commonsite ofsite of extranodalextranodal metastasis is the adrenalmetastasis is the adrenalgland, although adrenal insufficiency isgland, although adrenal insufficiency is

    distinctly uncommon. Lung cancer notdistinctly uncommon. Lung cancer notinfrequently presents initially as metastaticinfrequently presents initially as metastaticdisease, with the brain, bone, and liver alldisease, with the brain, bone, and liver allbeing common sites.being common sites.

    Carcinoma of the lungCarcinoma of the lung

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    Carcinoma of the lungCarcinoma of the lung

    Squamous Cell CarcinomaSquamous Cell Carcinoma

    Clinical FeaturesClinical Features:: Most squamous cellMost squamous cellcarcinomas present with symptomscarcinomas present with symptomsrelated to their bronchial origin,related to their bronchial origin,

    including persistent cough, hemoptysis,including persistent cough, hemoptysis,or bronchial obstruction, with the lastor bronchial obstruction, with the lastbeing accompanied by pulmonarybeing accompanied by pulmonary

    infections (recurrent pneumonias, lunginfections (recurrent pneumonias, lungabscesses) or atelectasis.abscesses) or atelectasis.

    Carcinoma of the lungCarcinoma of the lung

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    Carcinoma of the lungCarcinoma of the lung

    Squamous Cell CarcinomaSquamous Cell Carcinoma

    Clinical FeaturesClinical Features

    Extension of the tumor may causeExtension of the tumor may causecompression of the superior vena cava,compression of the superior vena cava,

    thereby resulting in severe venous andthereby resulting in severe venous andlymphatic congestion of the upper bodylymphatic congestion of the upper body(superior vena cava syndrome).(superior vena cava syndrome).

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    Carcinoma of the lungCarcinoma of the lung

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    Carcinoma of the lungCarcinoma of the lung

    SquamousSquamous Cell CarcinomaCell Carcinoma

    PathologyPathologyThe microscopic appearanceThe microscopic appearance ofof squamoussquamous cellcellcarcinoma is highly variable. The range ofcarcinoma is highly variable. The range ofdifferentiation extends from maturedifferentiation extends from mature

    squamoussquamous cells with keratin pearls to ancells with keratin pearls to ananaplasticanaplastic lesion recognized as being oflesion recognized as being ofsquamoussquamous cell origin only by electroncell origin only by electronmicroscopy andmicroscopy and immunohistochemicalimmunohistochemical

    examination. In wellexamination. In well--differentiated tumors,differentiated tumors,keratin often occurs as "pearls," which appearkeratin often occurs as "pearls," which appearas central, brightlyas central, brightly eosinophiliceosinophilic aggregates ofaggregates ofkeratin surrounded by "onion skin" layers ofkeratin surrounded by "onion skin" layers ofsquamoussquamous cells.cells.

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    ADENOCARCINOMAADENOCARCINOMA

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    epidemiology and risk factors:epidemiology and risk factors: notnot

    closely linked with cigarette smokeclosely linked with cigarette smoke macroscopic features:macroscopic features: typicallytypically

    peripheral, sometimes originating inperipheral, sometimes originating in

    areas of preareas of pre--existing lung scarringexisting lung scarring prognosis:prognosis: poor, because mostpoor, because most tumourstumours

    do not present signs of airwaydo not present signs of airwayobstruction (because of there peripheralobstruction (because of there peripherallocation) and therefore are highlylocation) and therefore are highlyadvanced before presentation;advanced before presentation;bronchioloalveolarbronchioloalveolar carcinoma has a goodcarcinoma has a good

    ro nosisro nosis

    histological features:histological features: 4 main4 main

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    histological patterns:histological patterns:

    acinaracinar (gland like spaces)(gland like spaces) papillary (fronds [yes this is a wordpapillary (fronds [yes this is a word --

    according to the Oxford dictionary, it canaccording to the Oxford dictionary, it canmean leafmean leaf--like] of tumor on thin septa)like] of tumor on thin septa)

    solid carcinoma withsolid carcinoma with mucinmucin productionproduction(poorly differentiated)(poorly differentiated)

    bronchioloalveolarbronchioloalveolar carcinoma (arises fromcarcinoma (arises fromClara cells or type IIClara cells or type II pneumocytespneumocytes;;distinctive in that it spreads through lungsdistinctive in that it spreads through lungsalong alveolar septa; often resembledalong alveolar septa; often resembledpneumonic consolidation; cells may secretepneumonic consolidation; cells may secretemucinmucin; tumor may be diffuse or focal); tumor may be diffuse or focal)

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    MESOTHELIOMAMESOTHELIOMA

    PathologyPathology

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    PathologyPathology

    Pleural mesothelioma. Grossly.Pleural mesotheliomacharacteristically compressesthe lungs.

    MESOTHELIOMAMESOTHELIOMA

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    PathologyPathology

    Microscopically, classicMicroscopically, classicmesothelioma exhibits a biphasicmesothelioma exhibits a biphasicappearance, namely epithelial andappearance, namely epithelial and

    sarcomatous patterns. Glands andsarcomatous patterns. Glands andtubules that resembletubules that resembleadenocarcinoma are admixed withadenocarcinoma are admixed withsheets of spindle cells that aresheets of spindle cells that are

    similar to a fibrosarcoma.similar to a fibrosarcoma.

    MESOTHELIOMAMESOTHELIOMA

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    PathologyPathology

    Pleural mesothelioma.A microscopic viewshows the sarcomatousand epithelial

    components of thetumor.

    secondarytumorsofthe lungsecondarytumorsofthe lung

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    y gy g

    etiologyetiology: any malignancy may reach lung: any malignancy may reach lung

    from lymph spread or vascular spreadfrom lymph spread or vascular spread

    clinical presentation:clinical presentation: is same as primaryis same as primarylesionslesions

    macroscopic features:macroscopic features: there are 3 common patterns:there are 3 common patterns:

    miliarymiliary pattern (many smallpattern (many small neoplasmsneoplasms))

    cannon ball pattern (one large, spherical, wellcannon ball pattern (one large, spherical, welldemarcated neoplasm)demarcated neoplasm)

    lymphangitislymphangitis carcimatosiscarcimatosis (spread along(spread along lymphaticslymphatics --white lines through lungs instead of black lines markingwhite lines through lungs instead of black lines markinglymphaticslymphatics; usually present with severe; usually present with severe dyspnoeadyspnoeabecause infiltration ofbecause infiltration of lymphaticslymphatics preventsprevents lymphaticslymphaticsremoval of fluid; is rapidly fatalremoval of fluid; is rapidly fatal

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    usually well circumscribedusually well circumscribed

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    yy

    may show features specific to tissue of originmay show features specific to tissue of origin(e.g. bone in lung suggestive of bone(e.g. bone in lung suggestive of bonemetastases, black suggests malignantmetastases, black suggests malignantmelanoma)melanoma)

    microscopic featuresmicroscopic features

    similar to tissue of origin (e.g. melanin suggestssimilar to tissue of origin (e.g. melanin suggests

    melanoma, bile suggestsmelanoma, bile suggests hepatocyteshepatocytes), although), althoughmost carcinomas look similar (e.g.most carcinomas look similar (e.g. squamoussquamouscell carcinoma primary to lung looks identical tocell carcinoma primary to lung looks identical tosquamoussquamous cell carcinoma primary tocell carcinoma primary to

    oesophagusoesophagus)) prognosis:prognosis: poor, except if local resection is possible (e.g.poor, except if local resection is possible (e.g.

    renal cell carcinoma often spreads to lung, therefore mayrenal cell carcinoma often spreads to lung, therefore mayonly effect one kidney, and cannon ball in lung, hence andonly effect one kidney, and cannon ball in lung, hence andoperation to remove kidney and part of lung is possible)operation to remove kidney and part of lung is possible)

    benign lung lesionsbenign lung lesions

    nonnon neoplasticneoplastic benign lesions:benign lesions:

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    nonnon--neoplasticneoplastic benign lesions:benign lesions:

    infectious (e.g. fungal, TB)infectious (e.g. fungal, TB)

    hamartomahamartoma

    inflammatoryinflammatory pseudotumourpseudotumour

    malformationmalformation

    neoplasticneoplastic benign lesions:benign lesions: carcinoidcarcinoid ((techniquelytechniquely this is invasive) (=this is invasive) (= neuroendocrineneuroendocrine

    tumors (5% of all bronchial lesions); protrude into lumentumors (5% of all bronchial lesions); protrude into lumenand often present with early airways obstruction and;and often present with early airways obstruction and;histologicallyhistologically cellscells maymayhave no abnormal features; 80% 10have no abnormal features; 80% 10year survival rate)year survival rate)

    salivary gland typesalivary gland type

    mesenchymalmesenchymal

    papillomapapilloma

    clear cell carcinomaclear cell carcinoma

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