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162 Cards in this Set
- Front
- Back
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Most obstructive lung diseases are due to decreased ______?
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Expiratory Airflow
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Where are anatomical lesions leading to obstructive pulmonary disease usually located?
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At the level of the terminal airways and/or alveoli
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Restrictive Pulmonary Disease is usually what type of disease?
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"Interstitial" (alveolar interstitium)
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Lung sizes typically seen w/ obstructive vs restrictive lung disease?
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Obstructive => large lungs
Restrictive => small lungs |
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Main Pulmonary Function Test results we need to focus on?
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FVC
FEV1 FEV1:FVC ratio |
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How does Obstructive lung disease affect pulmonary fxn tests?
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Decreased FEV1:FVC
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How does restrictive lung disease affect pulmonary fxn tests?
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FEV1:FVC is normal b/c both are decreased proportionally
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General Types of Obstructive Disease
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Chronic
Chronic w/ acute episodes |
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Examples of Chronic Obstructive Lung Diseases w/ acute epidsodes?
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Asthma
Constrictive Bronchiolitis Obliterans |
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Types of Chronic Obstructive lung diseases?
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COPD
Cystic Fibrosis Bronchiectasis |
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Two types of COPD?
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Emphysema
Chronic Bronchitis |
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Define Emphysema?
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PERMANENT enlargement of airspaces distal to terminal bronchioles accompanied by destruction of their walls
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Typical emphysema association?
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Rarely Unaccompanied by Chronic Bronchitis
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emphysema's definition is what type of definition?
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MORPHOLOGIC (pathologic)
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When is Emphysema not really emphysema?
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Overinflation
Interstitial |
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Types of overinflation mistakenly called emphysema?
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Compensatory emphysema (after pneumonectomy)
Senile E Obstructive E |
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Who get's emphysema?
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Present in 50% at autopsy, but most asymptomatic
SMOKERS Alpha-1 Antitrypsin Deficiency (but most are smokers too) |
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Subclasses of Emphysema?
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Centriacinar
Panacinar Distal Acinar |
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Panacinar E is typicall associated w/?
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A-1 AT Def
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Distal Acinar E is typically associated w/?
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Spontaneous Pneumothorax in younger people
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What part of lung is Centriacinar Emphysema most commonly found in?
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Apices
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Appearance of cross section of lung w/ centriacinar E?
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Big spaces mixed w/ normal spaces
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Gross association w/ Centriacinar Emphysema?
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Apical Bullae
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Which type of E is most severe?
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Panacinar
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Cross section appearance of panacinar E?
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all of it is big and bad
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Histologic Findings w/ Emphysema?
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Alveolar Wall Destruction
Bronchiolar Deformation (squiggly) |
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Pathogenesis of Emphysema?
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1. Smoking
2a. Increased Elastase 2b. Decreased Antielastase (aka A1 AT, so a def makes it worse) 3. Elastic Damage 4. Emphysema |
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Other organ typically affected by A1-AT def?
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Liver
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what happens to the liver in A1-AT Def? so?
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Cirrhosis
So Emphysema + Cirrhosis usually = A1-AT def |
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Definition of Chronic Bronchitis?
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Persistent, Productive Cough for at least 3 consecutive months in at least 2 consecutive years
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Type of Definition of Chronic Bronchitis?
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CLINICAL
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Subtypes of Chronic Bronchitis?
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Simple
Mucopurulent Asthmatic Obstructive |
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Who gets Chronic Bronchitis?
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Men>women
40-65 SMOKERS Urban Dwellers (smog...and smoking) |
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Gross Appearance of Chronic Bronchitis?
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Red Mucosa
Inc Vascularity in response to Inflammation |
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Histo of Chronic Bronchitis?
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Inc Mucus Glands
Inc Goblet Cells (mucin) |
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Difference between Blue Bloaters and Pink Puffers?
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Pink Puffers: Emphysema Dominant
Blue Bloaters: Chronic Bronchitis dominant |
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Appearance of Pink Puffers?
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Thin
Barrel Chest Prolonged expiration Hunched Posture Not Cyanotic |
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How do Pink Puffers usually present?
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Dyspnea
Wt Loss |
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How do blue bloaters present?
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Cough
Purulent Secretions Possibly Cor Pulmonale H/O Recurrent Infections Less pronounced dyspnea/resp drive Hypoxia |
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Appearance of Blue Bloaters
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Often Obese
Cyanotic |
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Common COPD Complications
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INFECTION:
Episodes of Decompensation (often due to infection) Gradual Deterioration of baseline |
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Types of Infections associated w/ COPD?
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Bacterial (most common)
Also susceptible to less pathogenic guys like Fungi (aspergillosis) or Legionalla Bronchitis, Pneumonia |
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What are some Late COPD Complications?
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Pulmonary Vascular Spasm
Pulmonary Hypertension Cor Pulmonale |
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What do COPD pts die from?
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Pulmonary Failure
Right HF Severe Infection |
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Kickers w/ Asthma Definition?
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Acute Episodes of reversible bronchoconstriction
Chronic, relapsing course Inc responsiveness to airway stimuli Due to Persistent Bronchial Inflammation |
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Types of Airway Stimuli in Asthma?
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Extrinsic
Intrinsic |
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examples of extrinsic asthma stimuli?
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atopic
allergic |
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examples of intrinsic asthma stimuli?
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non-atopic
idiosyncratic (aspirin, infection, exercise) |
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General Pathogenesis of Atopic Asthma?
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1. Sensitization
2. Early Phase (minutes) 3. Late Phase (hours) |
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What goes on during Early Phase?
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Mast Cells move to airway
Mucosal Ag Rxn Spasm, Edema, Mucus |
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what occurs w/ Late-Phase?
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Leukocyte recruitment
Epithelial Damage Spasm, edema, mucus some of same stuff as early phase just WORSE |
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Gross Appearance of Asthmatic Lungs?
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Overinflation
Atelectasis Mucus Plugs |
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Histo of Asthmatic Lungs?
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Increased Goblets and Mucus Glands
Increased Inflammatory Cells and other guys (masts, eos', PMN's, etc) |
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Histo Kickers for Asthma?
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Charcot-Leyden Crystals
Curschmann's Sprial |
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What are Charcot-Leyden Crystals and where do you find them?
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They're from eosinophils and found in the mucus
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what is a curschmann's spiral?
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whorl of shed epithelial cells + mucus
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Kicker cause of Constrictive Bronchiolitis Obliterans?
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INHALED DUSTS AND TOXINS
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How does Constrictive Bronchiolitis Obliterans present?
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Dyspnea
Obstructive PFT's |
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Imaging and Constrictive Bronchiolitis Obliterans?
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Normal or mildly hyperinflated CXR
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Prognosis and Rx for Constrictive Bronchiolitis Obliterans?
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Poor Response to Rx--> Poor Prognosis
gotta get a transplant |
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Main difference between Constrictive Bronchiolitis Obliterans and BOOP?
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Constrictive Bronchiolitis Obliterans: Constrictive
BOOP: Proliferative |
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General Types of Restrictive Lung Disease?
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Acute
Chronic |
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Examples of Acute Restrictive Lung diseases?
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ARDS/DAD
BOOP |
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Examples of Chronic Restrictive lung diseases?
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Idiopathic Interstitial Pneumonia
Drug-induced Environmental Immunologic Systemic diseases Angiitis and granulomatosis Lymphoproliferative |
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what is ARDS?
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Adult Respiratory Distress Syndrome
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What is ARDS?
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Rapid onset of severe resp. insufficiency
Usually a complication of another condition May progress to multiorgan failure Severe diffuse pulmonary congestion w/ hyaline membranes (like RDS in neonates) |
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what is DAD and what does it have to do w/ ARDS?
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Diffuse Alveolar Damage
DAD is the typical morphology for ARDS |
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Stages of ARDS?
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1. Lung Toxin
2a. Epithelial Injury 3a. Necrosis of Type 1 cells 2b. Endothelial Injury 3b. Leaky Caps 4. ACUTE STAGE: Edema, Hyaline Membranes 5. ORGANIZING STAGE: Alveloar Collapse, Fibrosis, Honeycomb Lung |
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When does the acute vs organizing stage of ARDS occure?
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Acute: 1st week
organizing: after that |
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Histo appearance of early ARDS?
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Thickened alveolar walls w/ hyaline membrane
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Histo appearance of mid phase ARDS?
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Type 2 pneumocyte hyperplasia
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Histo appearance of late stage ARDS?
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Honeycomb (scar tissue)
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What does BOOP stand for?
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Bronchiolitis Obliterans Organizing Pneumonia
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Kicker cause of BOOP?
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NOT INHALED TOXINS, CHEMICALS
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Two main kinds (at least that we need to know) of Idiopathic Pulmonary Fibrosis (IPF)
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UIP = Usual Interstitial Pneumonia
AIP = Acute Interstitial Pneumonia (hamman-rich) |
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Kicker w/ IPF pathogenesis?
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It looks like ARDS but it just takes a lot longer
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For ARDS and IPF who is responsible for the cell injury?
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B's and T's
Cytokines, TNF's, Proteases, etc |
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Histo appearance of UIP/IPF?
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Heterogenous
Early and Late Stage present on same slide |
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Numero Uno Cytotoxic Drug?
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Busulfan
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What is busulfan usually used for?
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CML
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what percent of pts on busulfan experience pulmonary issues? how many die?
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4%
a lot...and fast |
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Busulfan goes after what lung cells?
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Type 2 pneumocytes
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Main Types of Pneumoconioses?
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Asbestosis
Coal Workers' (CWP) Silicosis Berylliosis |
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Kicker for Pneumoconioses particles?
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SIZE MATTERS
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Which size particles are good? bad?
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1-5 microns are the most dangerous
5-10 don't make it all the way to the distal airways under 1 act like gases and go and and then back out |
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Kickers for how a particular patient will react to a toxic particle?
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Solubility, Concentration, Chemical Reactivity
Duration of Exposure Host Clearance Mechanisms |
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3 elements of Pneumoconiosis that make it so bad/
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Inflammation
Fibrogenic Toxic combine them and you get serious damage |
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what the crap is ferruginous and who cares?
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It means the Asbestos bodies stain w/ an Fe stain so you can tell what's going on
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Malignancy associated w/ asbestos?
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Mesothelioma
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Part of lung more commonly affected by asbestos?
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lower lobes
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How can solubility affect the level of damage a toxic agent causes?
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water-soluble particles are dissolved in the upper airway and removed-->lower airway protection
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what is Asbestos?
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Silicate
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Major types of Asbestos Fibers?
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Serpentine
Amphibole |
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Characteristics of Serpentine Asbestos fibers
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More common
curly flexible Shape gets them caught earlier in airways and removed by mucocilliary action |
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Characteristics of Amphibole Asbestos Fibers?
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much more pathogenic
esp for mesothelioma Rigid, Linear Shape lets them get farther into lungs |
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What exactly is an Asbestos Body and why are they bad?
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Amphibole fibers attract stuff (like tobacco carcinogens) and cause clumping on the actual fibers...
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Type of Fibrosis associated w/ Asbestosis?
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DIFFUSE (i.e. not nodular)
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Types of CWP's?
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Simple
Complicated |
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What are the Simple CWP lesions made of?
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Macules
Nodules |
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What are the macules made of?
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Interstitial peribronchiolar accumulations of dust
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types of macules?
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brown
black depends on type of coal |
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what are the nodules?
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they're palpable centers of fibrosis
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Outlook for Simple CWP?
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not really bad.
maybe mild emphysema |
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What is Complicated CWP normally associated w/?
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PMF
Progressive Massive Fibrosis |
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How big must lesions be to qualify as PMF?
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1-3cm...that's a big area of the lung to kill
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Other stuff seen w/ Complicated CWP?
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Lymphocytic Infiltration
Vascular Obliteration |
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What is Caplan's Syndrome?
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Rheumatoid Arthritis + CWP, Silicosis, or Asbestosis, but not Berylliosis
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Typical occurence w/ Caplan's?
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necrobiotic granulomatous nodules that resemble rheumatoid nodules
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Other occasional CWP and Silicosis associations?
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TB
Mycobacteria Infection |
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cancer and CWP/Silicosis?
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No relationship
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Which type of Silica is fibrogenic (bad)?
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Only the crystalline type (not the amorphous type)
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Jobs associated w/ Silicosis?
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Stonecutting
Sandblasting Quarry Work may contaminate other types of inhaled dusts |
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Typical presentation of silicosis?
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fibrotic nodules
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Types of Silicates?
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Asbestos
Talc Kaolin Mica |
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Histo appearance of silicosis?
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Concentrically arranged fibrous bands
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Silicosis complications?
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PMF
Caplan's Mycobacterial Infections |
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Silicosis under polarized light?
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Refractile
Birefringent |
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When does Acute Silicosis occur?
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High dose over a short period of time (1-3 years)
Sandblasters |
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Microscopic Acute Silicosis?
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looks like PAP
Pulmonary Alveolar Proteinosis |
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Types of Berylliosis?
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Acute (rare)
Chronic (usual) |
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Typical Result of Acute Berylliosis?
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ARDS/DAD
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Typical cause of Chronic Berylliosis?
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Occupational: Computer, Aerospace, Electronics
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Latency of Berylliosis?
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up to 15 years
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histo appearance
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Interstitial Fibrosis
Non-caseating Granulomas |
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Histo similarities of berylliosis?
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Sarcoidosis
Hypersensitivity Pneumonitis |
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Berylliosis Prognosis?
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Possibly Cancer
Possibly Pulmonary Failure Possibly remit, relapse, etc |
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What is GIP?
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Giant Cell Interstitial Pneumonia
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GIP AKA?
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Hard Metal Pneumoconiosis
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Cause of Hard Metal Pneumoconiosis?
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cobalt
Tungsten carbide diamond cutters |
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Who are the immunologic lung diseases?
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Hypersensitivity Pneumonitis
Pulmonary Eosinophilia Alveolar Hemorrhage Syndromes |
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Types of Alveolar Hemmorhage Syndromes?
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Goodpasture's
Idiopathic Pulmonary Hemosiderosis |
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Types of Hypersensitivity Pneumonitis?
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Farmer's Lung
Silo-Filler's Lung |
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Triad for Hypersensitivity Pneumonitis?
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Patchy Interstitial Pneumonia
BOOP Ill-defined, non-necrotizing interstitial granulomas |
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Another name for Hypersensitivity Pneumonitis?
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Extrinsic Allergic Alveolitis
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Typical Clinical Course of Hypersensitivity Pneumonitis?
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Delayed hypersensitivity reaction, but can be acute
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Typical agents responsible for causing reaction in Hypersensitivity Pneumonitis?
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Thermophilic Bacteria or Fungi
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Etiologic similarity and difference between Asthma and Hypersensitivity Pneumonitis
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Etiologically similar to allergic asthma.
But asthma hits on the small airways |
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Presentation of Chronic Hypersensitivity Pneumonitis?
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HARD TO DX
Dyspnea Dry Cough Fever Malaise Bilateral Reticulonodular CXR |
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Difference between Acute and Chronic Hypersensitivity Pneumonitis?
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Not much...just time
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% of Hypersensitivity Pneumonitis that die?
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5%
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Etiology of Sarcoidosis?
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UNKNOWN!!!
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Sarcoidosis is characterized by? what's the big deal?
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Non-Caseating Granulomas
not that helpful, so its a Dx of exclusion |
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What is Mikulicz's Syndrome?
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Sarcoidosis w/ involvement of major salivary glands and uvea
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Mortality Rate w/ Sarcoidosis?
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10-15%
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Wegener's involves?
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Upper and Lower RT's
Kidneys |
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Wegener marker?
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ANCA
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Wegener's biopsies show what on histo?
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Areas of dirty necrosis w/ undulating or stellate borders
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What causes the necrosis in wegener's?
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the vasculitis
can involves even tiny capillaries |
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What mimics Wegener's and must be distinguished microscopically?
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Angiocentric Lymphoma
the lymphoma cells can occlude small vessels => necrosis, BUT there is no invasion/destruction of vessel walls |
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Two restrictive lung diseases of uncertain origin we need to know?
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Pulmonary Alveolar Proteinosis (PAP)
Histiocytosis X |
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PAP on histo?
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Alveoli are filled w/ Eosinophilic, PAS-positive material
Material rich in surfactant phospholipids and proteins |
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PAP associations?
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may or may not be associated with any number of other disorders
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PAP prognosis?
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usually pretty good with Rx or lung lavage. Only a small small number die
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Risk w/ PAP?
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increased risk of Nocardia infection...maybe
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What is Histiocytosis X also known as?
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Eosinophilc Granulolma
or Langerhans Cell Histiocytosis |
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Main Histiocytosis X association?
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SMOKING
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What does Histiocytosis Imaging show?
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reticulo-nodular appearance
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Main Histological kickers with histiocytosis x?
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LANGERHANS Cells
Stellate Lesions eosinophils, lymphocytes, and plasma cells are usually present too |
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Stain for Langerhans cells?
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S100 protein
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Risks w/ Histiocytosis X?
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Malignancies
mainly lymphoma and lung carcinoma may just be due to smoking though |
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EM of Histiocytosis X?
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Langerhans/Birbeck Granules
aka X bodies |
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What are langerhans/birbeck granules?
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pentilaminar structures that kinda look like tennis rackets
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