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55 Cards in this Set
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chronic functional abnormality (measured by physiologic tests) chracterized by diminished ability to expire air
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chronic airflow obstruction : CAO
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synonyms for CAO
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COPD
chronic airflow limitation CAL chronic obstructive lung disease COLD |
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COPD definition
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clinical term that encompasses several specific disorers that have diff clinincal manifestations, pathologicfeatures, reuirements for therapy, and prognoses
all are characterized by persistent limitation of airflow - usually assessed by tests ofexpiratory airflow : FEV1 |
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airflow determined by
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pressure applied and resistance encountered
like flow in any hydraulic system inlung, pressure derives from elasic rcoil of lung reduced recoil pressure is generally considered the chief defect in emphysema |
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what results from narrowing of airways
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increased resistance
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COPD clinical term encompasses
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a spectrum of disorders that range from asthmatic bronchitis to chronic bronchitis to emphysema
COPD 4th leading cause of death in US today major cause of morbidity, affecting as many as 30 mill americans |
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most important cause of COPD
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cig smoking
esp chronic bronchitis & emphysema industrial causes & air pollution have also been implicated,but association dwawrfed by overwhelming association with cig smoking spectrum of disease in COPD group likely reflects the spectrum of smoking damage inflicted on resp tract at various levels: bronchi, bronchioles, acini |
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clinical chronic bronchitis
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persistent cought with sputum production occuring on most days for at least 3 months of the year for at least 2 consecutive years (chronic excess secretion of mucus into bronchial tree), without another specific cause for mucus hypersecretion
- such as tb, cancer, etc ambiguous term, but embedded in clinical literature - more of a clinical symptom than a speceif disease entity primarily disorder of smokers |
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chronic bronchitis pathology
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chronic mucosal irritants such as smoke, air pollutioin are thought to damage airway mucosa and result in nonspecific increase in secretion of bronchial mucus from both the surface goblet cells and quantitatively more important submucosal glands
damge to mucociliary blanket predisposes thses pts to recurrent infections, which add to mucosal drainage |
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large airways in chronic bronchitis
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boggy, hyperemic bronchial mucosa
hypertrophy of submucosal glands: increased Reid index increased goblet cells epithelial metaplasia/dysplasia wit focal loss of ciliary lining varying degress of mucosal inflammation |
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chronic bronchitis in small airways
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goblet cell metaplasia
mucous plugging inflammation fibrosis pigmented macs |
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term referring to variety of changes found in small (<2mm) peripheral airways of pts with COPD
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small airways disease
sig overlap with findings in chronic bronchitis & emphysema since almost al pts wth these disorders will also have varying degress of small airways disease |
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small airways contribution to total airway R
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under normal circumstances, account only a small proporition (10-15%) of total airway R
in COPD major increase in R appears to occur in smallairways much evidence that tests of small airways funciton become abnormal even before FEV falls to abnormal levels, reflecting damage to these membranous bonchioles in smokers |
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pathology of small airways disease
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bronciholitis - consistent finding in both mild & severe COPD (emphysema & chronic bronchitis)
goblet cellmetaplasia mcuous plugging inflammation fibrosis pigmented macs |
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small airway disease pathogenesis
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smoking most important epidemiologic factor in development
although small # of cases may be associtad with severe infections, inhalation of toxic fumes, RA or diffuse panbronchiolitis |
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emphysema anatomic definition
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abnormal, permanent enlargement of airspaces distal to terminal bronchiole (acinar tissue), accompanied by destruction of resp tissue, witout sig fibrosis
classified by way the acinus/lobule involved 4 patterns recognized: centriacinar, panacinar, distal acinar, irregular |
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centriacinar emphysema
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proximal acinar, centrilobular
resp bronchioles dominantly involved upper lung zones generally more severly affected than lower zones strong smoking association most common form of emphysema * |
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panacinar emphysema
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panlobular
involves entier acinus more or less uniformly lower and anterior lung zones generally more severely affected this pattern may be associated with familial alpha 1 antitrypsin def associated with smoking |
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distal acinar
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paraseptal, subpleural
alveolar ducts dominantly involved leastt common form predominantly foundalong post or ant margins of upper lung zones classically associated with spontaneous pnumothroax of young adults, esp tall, young adult males |
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irregular emphysema
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scar
irregular acinar involvement due to adjcent scarring |
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what 2 emphysema forms are most frequently associated with clinical evidence of COPD
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centriacinar & panacinar
only rarely will paraseptal or irregular be associated with clinical COPD many cases with COPD & emphysseama re mixes of centri & pan lung volumes (TLC, RV) often increased, reflecting pathologic increase in vol of lung associated with loss of lung elastic recoil |
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antiprotease hypothesis/oxidant antioxidant hypothesis
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presently thought that destructive emphysematous changes in lung parenchyma associated with cig smoking are due to unrestrained proteolytic activity that destroys lung CT
oxidant antioxidant imbalance prob also contributes |
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clinical features of emphysema
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usually clinically silent until 20-30% of lung parenchyma destroyed
pts are symptomatic generally present with features of COPD - dyspnea - prolonged esxp phase - CXR: large lungs with low diaphragms - loss of lung elastic recoil - decreased DLCO great majority are smokers most symptomatic pts are > 50-60 yo if onset < 40 yo think about familial alpha 1 antitrypsin def |
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causes of death in emphysema
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resp failure
copulmonale pneumothroax coronary artery disease |
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bronchiectasis anatomic definition
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permanent, abnrormal dilation of bronchi (and bronchioles)
usually resulting in recurrent, chornic necrotizing infections & obliteration of distal airways |
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classificaitons of bronchiectasis
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postinfective: adenovirus, measles, pertussis, tb, staph, intralobular sequestration
bronchial obstruction: foreign bodies, tumors, mucous plugs irritants: ammonia aspiration: heroin addicts hereditary abnormalities: CF, ciliary dyskinesia congenital anomalies: hypogammaglobulinemia, defective pmn's, macs idiotpathic: 50% cases in US |
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in 70% of bronchiectasis cases, a history of
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severe bronchopulmonary infection in childhood
evidence to suggest that decreased incidence of bronchiectasis in industrialized worls is related to appropriate antibiotic therapy obstruction and/or infection are major conditions assoicated with bronchiectasis |
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bronchiectasis pathogenesis
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sig airway damage with obliteration of disttal bronchi & bronchioles leads to atelectasis
mucous pooling, ineffective cough, damage to mucociliary blanket lead to infection, which further damages airway |
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bronchiectasis clinical
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productive cough
often purulent foul smelling sputum intermittent infections fever hemoptysis |
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bronchiectasis pathology
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distribution varies with cause
- hereditary defects often diffuse ; CF - postinfective & idiopathic are often loclized to lower lobes (esp basal segments) and elft more common than rigth - asthmatics- upper lobes more commonly involved. Distal parenchyma atelectatic, fibrotic, inflamed. Extensive inflammation with mucosal ulceration, destruction of airway walls |
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generic term for large group of pulmonary disorders with several clnical features in common
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diffuse infiltrative (interstitial) lung disease
some of these disorders are acute (diffuse alveolar damage) with a clinical course measured in days to weeks however, most are chronic with clnical courses that transpire overmonths to years - most of these chornic entities are generally associated with varying degress of interstitial pneumontiis and/or fibrosis and many have minor intra alveolar component terminology for this group of dissease is confusing b/c various terms have been applied to same diseases and some of the cliniical terms include more than one pathologic abnormality (idiopathic pulmonary fibrosis) |
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clinical features of ILD (chronic diffusse interstitial (restrictive) diseases)
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dyspnea, wt loss
physiologic studies: restrictive pattern (filling of lung imparied, TLC and RV are decreased) hypoxia, low CO diffusing capacity, decreased complaince. hypercapnea usually absent CXR: diffuse reticular and/or nodular pattern common, small lung vol |
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dx and etiology of ILD
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over 150 causes of diffuse Infiltrative lung disease
due to diverse causes, personal & fam medical histories, drug & chemical exposure history, careful occupational history, as well as recreational activities, hobbies, travel history and social history may be very importnat in sorting out this nonspecific clinical presentation and hopefully will help to establish dx and etiology of ILD |
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etiologies of diffuse ILD
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environmental disease 25%
- allergic alveolitis, pneumoconiosis, toxic gases sarcoidosis: 21% chronic idiopathic interstitial pneumonies 13% - UIP, DIP, LIP CT disorders 8% - progressive systemic sclerosis, systemic lupus, rheumatoid dz pulmonary vascular disease 6% - arteritis, veno occlusive dz, emboli infections 3% - miliary tb, fungi, parasites, viruses malignancies 3% over 100 disorders account for remaining cases! |
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fibrosing disorders include
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usual interstitial pneumonia UIP
idiopathic pulmonary fibrosis most common of chornic idiopathic interstitial pneumonias nonspecific interstitial pneumonia NSIP organizing pneumonia (OP)/bronchioltiis obliterans organizing pneumonia (BOOP) CT disorders |
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UIP clinical
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insidious onset of dyspnea over multiple years
progressive, chronic downhill course avg survival 3-5 yrs 50-70 yr old at presentation similar pattern in : CT diseases (RA, SLE, PSS), chronic hypersensitivity pneumonitis, asbestosis |
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UIP pathology
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small lungs with fibrosis more marked peripheral and basal
inflammatory and fibrosing lesions with marked histologic variability interstitial inflammation, fibrosis with focl alveolitis (fibroblastic foci) end stage honeycomb lung in areas, esp lower zonal, subpleural |
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UIP pathogenesis
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idiopathic: precise triggering mechanism initiating inflammatory/fibrotic process is not known
immune complexes, autoantibodies seen in some pts some evidence that genetic factors may also play a role |
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NSIP clinical
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45-55 yr old at presentation
dyspnea/cough of several months duration bilateral interstitial infiltrates lack honeycomb change and fibroblasttic foci of UIP |
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NSIP pathology
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patterns: cellular (inflammation but little fibrosis) and fibrosing (inflammation & fibrosis)
both have relatively uniform intterstitial infiltrate of lymphs, plasma cells |
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NSIP prognosis
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better than UIP
cellular better than fibrosing |
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organizing pneumonia
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organizing bronchiolar and associated lveolar exudates: loose, fibrous airspace lesions
- masson bodies BOOP pattern can have many etiologies: infection (viruses, bacteria), toxic inhalants/drugs, collagen vascular disease (RA), aspiration, bronchial obstruction, idiopathic |
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idiopathic form of op
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specific clinicopathologic entitiy: COP
usually middle aged adult with cough, dyspnea of 4-6 wks duration often following flu like illness |
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OP CXR
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patch, sometimes migratory, often bibasilar interstitial and airspace infiltrates
ground glass appearance |
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many idiopathic cases of OP will respond to
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steroids
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CT fibrosiing disorders
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RA: 30-40% have lung involvement
- pleuritis, UIP like pattern, rheumatoid nodules, pulmonary HTN, follicular bronchiolitis systemic sclerosis (scleroderma): NSIP & UIP patterns (variable fibrosis) lupus erythematosus: acute lupus pneumonia (alveoltiis, interstitial inflammation, edema), NSIP, acute hemorrhage, pleuritic variety of drugs & radiation mayy also causse lung reactions that vary from acute pneumonitis to dense fibrosis |
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granulomatous disorders include
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sarcoidosis
hypersensitivity pneumonitis : extrinsic allergic alveolitis |
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sarcoidosis
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multisystem granulomatous dissease of unknown etiology that frequently involes the lung & hilar LN's
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sarcoidosis clinical
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usually young adults
black : whites = 10:1 genetic presdisposition: HLA-A1, HLA-B8 multisystem disease that may involve LNs, skin, eyes, liver, CNS, plus other systems some are anergic and/or have polyclonal gammopathy dx based on combo of clinical, radiographic and lab findings combined witth compatible histologic pattern |
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sarcoidosis pathology
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non necrotizing granulomas (often obtained by transbronchial biopsy) distributed in lymmphangitic pattern - dx of exclusion
must r/o other causes of granulomatous inflammation - mycobacteria, fungi, verryliosis |
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sarcoidosis prognosis
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70% eventual recovery with minimal to no loss of lung function
20% have some permanent loss of lung function 10% may die of their disease - progressive pulmonary fibrosis, cor pulmonale, heart and/or CNS involvement |
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hypersensitivity pneumontiis
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type of immunologic rxn in lung o variety of inhaled antigens (3 major categories):
- thermophilic bacteria - molds - animal proteins clinical entities that fit into this pathologic entity are numerous and often named according to clinical circumsttances of exposure (pigeon breeders lung, farmers lung -thermophilic actiniomycetes -, muschroom pickers lung, duck fever, etc |
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hypersensitivity clinical
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dyspnea, fever, chills, cough develop 4-6 hrs after exposure to offending agent
if no further exposuree- symptoms resolve over the next 12-24 hrs reexposure generates new wave of symptoms and repeated exposures lead to interstitial fibrosis wtih permanent lung injury chronic, low dose exposure ma present with more insidious cocuse |
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hypersensitivity pneumontisi pathology
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patchy, peribronchiolar interstital neumonitis with predominnalty chronic inflammatory cells +/- granulomatous inflammation
pathcy airspace inflammation |
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hypersenstiviity pneumontiis pathogenesis and tx
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most likely involves combo of type iii (IC mediated or arthus) and type IV (delayed or cell mediated) immune response
skin tests, preciptating antibodies ma be helpful in proper clinical setting tx: prevent exposure to offending agent! |