Pulmonary Pathology Part 2 Flashcards
What are some components of restrictive lung disease?
- Volume restriction
- FEV1/FVC normal
- FVC reduced
What are some components of obstructive lung disease?
- Decreased flow
- Low FEV1/FVC ratio
What is the key fact of restrictive lung disease?
- REDUCED VOLUME
What is the key fact of obstructive lung disease?
- AIR TRAPPING
Where does the loop shift in flow volume loops with obstructive lung disease?
- To the left
- Lung volume is greater than normal but FEV1 is greatly reduced
Where does the loop shift in flow volume loops with restrictive lung disease?
- To the right
- Lung volume is less than normal and both FEV1 and FVC are reduced
What is the most common cause of obstructive pulmonary disease?
- COPD/Chronic bronchitis due to smoking
What happens in chronic bronchitis?
- Mucous gland hyperplasia causing damage to airway epithelium
What does chronic bronchitis present as?
- Persistent cough with sputum production for 3 months out of 2 consecutive years (Diagnostic)
What are some complications of chronic bronchitis?
- Bronchiectasis
- Death from respiratory infection
- Squamous metaplasia/dysplasia/carcinoma
What is emphysema?
- Alveolar ducts are narrowed causing alveolar sacs to collapse and develop holes
What does emphysema look like on clinical presentation?
- Enlarged lungs on CXR
- Flattened diaphragm
- Barrel chest with increased AP diameter (same diameter all around chest)
- Diminished breath sounds with prolonged expiratory wheezes
What do PFTs show in patients with emphysema?
- Obstructive pattern
What is good description of someone with chronic bronchitis?
- Blue bloater
What is a good description of someone with emphysema?
- Pink puffers
Is emphysema reversible or irreversible?
- Irreversible
What does a1-antitrypsin do in the lungs?
- Coats lungs, protecting them from neutrophil elastase
What happens physiologically in a1-antitrypsin deficiency?
- A1-antitrypsin stays in the liver rather than going to lungs causing liver damage
- Due to the lack of a1-antitrypsin, the lung is susceptible to damage from neutrophil elastase
What does the lung look like in a1-antitrypsin?
- Panacinar or pan lobar emphysema
What are some other types of emphysema?
- Spontaneous PTX
- COPD
- Localized
- A1-AT deficiency
What gene encodes for a1-AT?
- Pi gene on chromosome 14
What is the Z allele associated with in a1-AT deficiency?
- Decreased circulating a1-AT
What is seen in individuals that are homozygous for PiZZ?
- Markedly decreased a1-AT with a majority developing panacinar emphysema
How do you diagnose a1-AT deficiency?
- Serum testing for a1-AT
What are some complications of emphysema?
- Respiratory failure
- Pneumothorax with lung collapse
- Coronary artery disease
- Right heart failure
What kind of emphysema does a spontaneous PTX present with?
- Distal acinar or paraseptal
What kind of emphysema does COPD present with?
- Centrilobular, centriacinar, or proximal acinar
What kind of emphysema does localized present with?
- Irregular
What are the three components of asthma?
- Recurrent airway obstruction with a reversible component
- Airway hyper-responsiveness
- Airway inflammation
What is atopic asthma classified as?
- 2/3 of all patients
- May be at any age, typically childhood
- Family history of asthma
- Elevated IgE levels (eosinophils, mast cells, lymphocytes)
- Triggers may include a variety of allergens
What is non-atopic asthma classified as?
- 1/3 of all patients
- Often older patients
- Typically normal IgE levels (T-lymphocytes, neutrophils)
- Triggers may include cold, exercise, infection
What kind of hypersensitivity reaction is atopic asthma?
- Type 1
What cells are increased in atopic asthma?
- Eosinophils
- Mast cells
- Lymphocytes
What cells are increased in non-atopic asthma?
- T-lymphocytes
- Neutrophils
What is the pathogenesis of asthma?
- Allergen is presented to dendritic cell
- Th2 cell releases IL4 and IL5
- IL4 recruits IgE B cells which help get mast cells
- IL5 recruits eosinophils which release granules and mediators
What mediators cause the bronchoconstriction in asthma?
- Leukotrienes C4, D4, E4
- Histamine
- Prostaglandin D2
- Acetylcholine
What mediators cause the mucus secretion in asthma?
- Leukotrienes C4, D4, E4
What mediators cause the increased vascular permeability?
- Leukotrienes C4, D4, E4
What mediators cause the recruitment of inflammatory cells?
- Interleukins
What happens in airway remodeling?
- Progressive structural changes to airways with characteristic histologic findings
What is seen histologically in airway remodeling?
- Fibrosis
- Smooth muscle hyperplasia
- Increased goblet cells and submucosal glands
What is status asthmaticus?
- An unremitting, potentially fatal asthma attack
- Causes a bronchial occlusion by thick mucus
What is a “Curschmann’s spiral”?
- A coiled thick mucus plug
What are some genetic associations with atopic asthma?
- Associated with seasonal allergies and eczema
- May be linked to various alleles controlling factors like IgE, cytokines, adrenergic receptors
What are some environmental associations with atopic asthma?
- Disease of industrialized societies (pollution and lack of allergen exposure at early age)
- Early infection
What is aspirin sensitive asthma?
- Due to the COX blocking effect of aspirin, all of the arachidonic acid is pushed to make leukotrienes C4, D4, E4 which cause bronchoconstriction
What is aspirin associated asthma associated with?
- Nasal polyps and recurrent rhinitis (Samter’s triad)
What is bronchiectasis?
- Necrotizing inflammatory response
- Usually the end stage process of multiple processes (Infection or Obstruction)
What can bronchiectasis be seen in?
- Allergic bronchopulmonary aspergillosis (ABPA)
- Cystic fibrosis
- Chronic infection (TB)
- Primary ciliary dyskinesia
What is kartagener’s syndrome?
- Dysfunction of dynein arm of microtubules
What is the triad seen in kartagener’s syndrome?
- Sinusitis
- Bronchiectasis
- Situs inversus
(will often see male infertility too)
What is primary ciliary dyskinesia?
- Microtubules are vital to motility of various cell populations (cilia and flagella)
What is allergic bronchopulmonary aspergillosis (ABPA)?
- Exaggerated hypersensitivity response to aspergillus infection overlying chronic lung disease
What is seen in ABPA?
- Background of asthma or cystic fibrosis
- Increased IgE on serum testing
- Positive skin test
- Thick dark mucus in bronchi
- Associated with bronchiectasis in advanced disease
