ICL 3.1: Pathology of Obstructive Lung Diseases Flashcards

Question

what is the required morphologic criteria needed to diagnose emphysema?

Answer 1

1. abnormal permanent airspace enlargement 2. involvement distal to terminal bronchiole 3. airspace wall destruction** 4. lack of significant interstitial fibrosis

Answer 2

more common and severe in males clear association with cigarette smoking generally does not become disabling until the 4th-7th decades but ventilatory deficits may make their first appearance decades earlier

Answer 3

it's based on the anatomic distribution of the damage in the lobule 1. centriacinar = proximal acinar is involved 2. panacinar = the whole acinar is involved 3. paraseptal = subpleural and upper half of the lungs is involved

Answer 4

centrilobular accounts for > 95% of the cases clinically significant airway obstruction is seen in both centriacinar and panacinar emphysema

Answer 5

it starts in the respiratory bronchioles and destroys the central (proximal) parts of the acinus both emphysematous and normal areas exist in the same acinus and lobule predominately involves the upper lobes; especially apical segments it's more frequent in heavy smokers so it's often seen in associated with chronic bronchitis

Answer 6

associated with alpha-antitrypsin deficiency which normally inhibits proteases that break down elastin in the walls of the alveoli more common in lower lobes and in the anterior margins of the lungs so it's most severe in the lung bases --> it's most common in the bases because that's where there's the most blood flow and the blood is what brings in all the inflammatory cells and neutrophils are sequestered in the lungs in the lower lobes and they release elastase! it affects the ENTIRE acinus but there's initial involvement of the peripheral alveoli and alveolar ducts

Answer 7

centiracinar 1. upper lobes 2. cigarette smoking 3. involves the central portion of the acinus panacinar 1. lower lobes 2. alpha antitrypsin deficiency 3. involves the entire acinus

Answer 8

protease-antiprotenase theory proteases are enzymes that digest proteins alveolar wall destruction results from an imbalance of elastase, a protease, and alpha-antitrypsin, an antiprotenase

Answer 9

smoke particles impact in small bronchi and bronchioles and form ROS that inactivate antiproteases while increasing inflammatory cytokines then an influx of neutrophils and macrophages which leads to increased elastase more common in the upper lobe because it's less perfusion and there's less α1-AT and smoke rises

Answer 10

α1-AT deficiency throughout acinus neutrophils are normally sequestered in lungs, more in lower than upper lobe -- neutrophils release elastase! so that's why there's more damage in the lower lobes with apnacinar emphysema more common in the lower lobe – more perfusion

Answer 11

dyspnea is the initial symptom --> symptoms appear when at least 1/3 of the parenchyma is effected not too much cough or sputum; you'll only see it if there's accompanying chronic bronchitis when there's severe emphysema, you'll see marked inflation of the lungs, barrel chest, and flattened diaphragm you'll also see weight loss because of how hard it is to breath and enlargement of accessory muscles of respiration

Answer 12

1. respiratory acidosis and coma 2. right sided heart failure 3. massive lung collapse second to pneumothorax some of the expanded alveoli can rupture and release air into the pleura

Answer 13

pink puffers they have dyspnea because of the low pressure during expiration that will cause their lungs to collapse without elastin; so they pinch their nose and purse their lips and sit forward to try and increase the pressure in their lungs so that their bronchioles don't collapse as much and this is where they get the name pink puffers! they have to use accessory muscles of respiration to do this (so check for their enlargement) and they have prolonged, forced expiration so they lose a ton of weight because of how hard it is to breathe oxygen saturation is maintained at near normal levels from over inflation of the lungs so they're not cyanotic, they remain pink

Answer 14

alpha1-antitrypsin deficiency the alpha1 produced in the liver can't leave the liver so it destroys hepatocytes and you get cirrhosis on top of the emphysema

Answer 15

α1-AT is produced by liver and inhibits a variety of proteases (specifically elastase) it accounts for 90% of antiprotease activity in the blood and without it, neutrophil elastase digests the human lung production of alpha-AT depends on a pair of co-dominant alleles – Pi (protease inhibitor) gene on chromosome 14*. think α1-AT deficiency when individual is diagnosed with emphysema at < 40 yrs of age * Normal phenotype Pi MM but alpha-antitrypsin deficiency has phenotype Pi ZZ

Answer 16

paraseptal emphysema hyper resonance = lots of air in the pleural cavity sharp pain = pleura

Answer 17

involves predominantly the distal portion of the acinus usually more severe in upper half of lung most prominent near the pleura, along the lobular connective tissue septa and at the margins of the lobules **associated with spontaneous pneumothorax in young male adults

Answer 18

alpha1-antitrypsin deficiency

Answer 19

enlargement of air spaces WITHOUT destruction so it's NOT emphysema

Answer 20

distension of airspace's in the preserved lung after unilateral pneumonectomy or lobectomy

Answer 21

patient breaths in but can't get any of the air out may be life threatening usually occurs when there's a tumor or we inhale a foreign body

Answer 22

congenital lobar overinflation in infants due to hypoplasia of bronchial cartilage without cartilage in the bronchioles, you can't exhale because the bronchioles are flacid and they'll collapse during expiration and trap air

Answer 23

chronic, relapsing inflammatory disorder characterized by tracheobronchial hyper-reactivity leading to paroxysmal, reversible airway narrowing (episodic bronchoconstriction) two major components: 1. chronic airway inflammation 2. hyperactive bronchial tree relapsing = comes and goes it's reversible! so it doesn't last

Answer 24

10% of kids 5% of adults 1/2 the cases develop i patients under 10 years old there has been a significant increase in the incidence of asthma in the Western world in the past 3 decades

Answer 25

1. extrinsic (immune) | 2. intrinsic (non-immune)

Answer 26

caused by exposure to extrinsic antigen caused by type I IgE hypersensitive; usually begins in childhood often there's a family history of allergy or associated allergies like dust, pollen, food cause by: 1. atopic (allergic) – most common 2. occupational 3. allergic bronchopulmonary aspergilliosis

Answer 27

non-immune mechanism usually in adults often there's no family history of allergy or associated allergies most common cause is respiratory tract infections like viruses caused by: 1. drug induced (e.g. aspirin = AERD) 2. pulmonary infections, especially viral 3. inhaled irritants 4. stress 5. exercise 6. cold

Answer 28

intrinsic asthma it is thought that virus-induced inflammation of respiratory mucosa lower the threshold of the subepithelial vagal receptors to irritants

Answer 29

1. leukotrienes, C4, D4, and E4 2. acetylcholine 3. histamine 4. prostaglandins these are all associated with asthma!

Answer 30

prostaglandins --> prostacyclin --> vasodilation and increased vascular permeability PGD2 causes vasodilation acting via the prostaglandin (DP) receptor on vascular smooth muscle, and myocontraction acting via the thromboxane (TP) receptor on bronchial smooth muscle they play a role in the bronchospasm associated with asthma

Answer 31

produces leukotrienes like C4, D4, E4 which all cause bronchospasm and increased vascular permeability they play a role in the bronchospasm associated with asthma

Answer 32

1. leukotrienes, C4, D4, and E4* 2. acetylcholine 3. histamine* 4. prostaglandins 5. platelet activating factor* which causes platelet aggregation and release of histamine and serotonin from their granules 6. cytokines, IL1, TNF, IL6 7. major basic protein of eosinophils which cause epithelial damage in the bronchus

Answer 33

1. ADAM 33* 2. IL4 receptor gene variants 3. YKL-40* 4. class II HLA alleles

Answer 34

ADAM33 belongs to a subfamily of metalloproteinases,mainly collagenases ADAM33 is expressed on lung fibroblasts and bronchial smooth muscles polymorphism accelerates proliferation of bronchial smooth muscle cells and fibroblasts which leads to narrowing of the bronchus the above contributes to bronchial hyperactivity and subepithelial fibrosis that is associated with asthma

Answer 35

IL4 receptor gene variant are associated with atopy and increased age and asthma atopy refers to the genetic tendency to develop allergic diseases such as allergic rhinitis, asthma and atopic dermatitis (eczema)

Answer 36

it's a specific chitinases that is associated with asthma it triggers TH2 inflammation serum levels of YKL-40 correlates with the severity of asthma

Answer 37

aspirin and NSAIDS inhibit COX pathway of arachidonic acid metabolism without effecting lipoxygenase pathway this leads to decreased prostaglandin synthesis and increased production of leukotrienes = bronchoconstriction and increased permeability urinary LTE4 levels are a marker of systemic cysLT production and are increased in AERS

Answer 38

1. recurrent rhinitis/sinusitis 2. nasal polyps 3. asthma and often urticaria

Answer 39

1. mucous plugs in the lumen 2. elevated eosinophil count* 3. bronchial wall changes = airway remodeling! - thickened basement membrane - edema and inflammatory infiltrate of the bronchial wall, prominence of eosinophils and mast cells - hypertrophy of submucosal glands - hyperplasia of goblet cells in the mucosa - hypertrophy of smooth muscle

Answer 40

1. charcot-leyden crystals formed from eosinophil major basic protein -- look like swords 2. Curschmann spirals strands of mucous to which epithelial cells may be attached/trapped

Answer 41

1. primarily muscular contraction 2. edema and inflammatory infiltrate eosinophils and mast cells are the major components of the inflammatory infiltrate eosinophils are destructive because of the major basic protein while mast cells release histamine

Answer 42

attacks that last several hours with wheezing, respiratory distress followed by prolonged cough and copious sputum it's a lot of recurrent attacks that are more disabling rather than lethal and the disease can disappear spontaneously

Answer 43

incredibly severe bronchoconstriction associated with asthma that can last several days they can die from ventilatory compromise

Answer 44

chronic necrotizing infection of the bronchi and bronchioles leading to irreversible dilation of these airways from destruction of the muscle and elastic tissue usually associated with hereditary conditions or post infectious conditions dilatation must be permanent

Answer 45

bronchiectasis low immunoglobulins and CD4 cells with immunocompromised people! makes them susceptible to bacteria foul smelling is from pus in the bronchi

Answer 46

1. cystic fibrosis 2. immunodeficiency states 3. Kartagener's syndrome

Answer 47

any infection or objstruction that damages and permanently enlarges and thickens the bronchioles such as: 1. hereditary conditions --> CF, immunodeficiency states, Kartagener's 2. posinfectious conditions --> necrotizing pneumonia 3. bronchial obstruction 4. post-transplant

Answer 48

1. infection --> inflammation --> necrosis and fibrosis --> dilated airways 2. obstruction --> normal clearing mechanisms impaired --> airway inflammation and pooling of bronchial secretions obstruction and infection are important in the development of bronchiectasis

Answer 49

generally effects the lower lobes bilaterally most severe in the distal bronchi and bronchioles airways may be dilated 4x normal size! they get so big that the bronchi can generally be followed to the pleural surface on gross examination transected bronchi look like cysts filled with mucopurulent material/pus

Answer 50

ACTIVE DISEASE 1. acute chronic inflammatory exudate in walls of bronchi and bronchioles 2. desquamation of the lining epithelium 3. necrosis and ulceration of the wall 4. squamous metaplasia* 5. abscess formation* CHRONIC DISEASE 1. fibrosis of the walls of the bronchi and bronchioles 2. subtotal or total obliteration of bronchiolar lumen

Answer 51

1. foul smelling sputum* 2. hemoptysis 3. paroxysmal cough worse in the morning from changing position draining pus into bronchi 4. dyspnea and wheezing* 5. pneumonia, lung abscess 6. fever, weight loss, weakness 7. cor pulmonale*, metastatic brain abscess, amyloidosis

Answer 52

increased resistance to airflow due to partial or complete obstruction from trachea to respiratory bronchioles

Answer 53

reduced expansion of lung parenchyma with decreased total lung capacity

Answer 54

asthma EBP damages epithelium

Answer 55

emphysema alpha1-antitrypsin deficiency phenotype

Answer 56

asthma made of major basic protein from eosinophils

Answer 57

extrinsic asthma

Answer 58

bronchiecstasis

Answer 59

bronchiecstasis

Answer 60

alpha1-antitrypsin deficiency

Answer 61

Curschmann spirals asthma