CUMULATIVE PATHOLOGY Flashcards
What kind of pulmonary edema would be caused by an obstruction of pulmonary veins?
Hemodynamic pulmonary edema
Why would a person with emphysema get heart failure?
Because the lung disease can cause pulmonary hypertension = cor pulmonale
What is the cause of the type of emphysema that mostly affects the lower parts of the lungs?
This is Panacinar emphysema and is caused by homozygous A1AT deficiency
Describe how endothelial damage seems to occur in Wegener’s:
By having c-ANCA target the neutrophils (Neutrophil Proteinase-3) it ACTIVATES the neutrophil which selectively targets endothelium
What is the arterial supply/venous drainage of pulmonary sequestrations?
Both extralobar and intralobar are supplied by aorta NOT pulmonary artery, and drained by azygous system
Explain why a person with a saddle embolus has electromechanical dissociation (i.e. PEA)?
Because their heart generates a rhythm but no blood can get past the pulmonary trunk, so no blood goes systemically either producing pulseless electrical activity.
When is pulmonary hypoplasia fatal?
If the disease is bilateral
What is the major complication of a bronchogenic cyst? Another name for these?
Infection; Foregut cyst
How far down do the most inferior portions of a lung normally go?
They are usually level with the cardiac apex
What is Anti-DNA topoisomerase I antibody useful for, diagnostically?
Scleroderma
Regarding Wegener’s: 1) What is the typical lung lesion? 2) What is the typical renal lesion?
1) Necrotizing granulomatous alveolitis 2) Necrotizing glomerulonephritis
What are the late microscopic findings in chronic bronchitis (3)?
Goblet cell hyperplasia, increased Reid index, and squamous metaplasia
How does Wegener’s differ from Goodpasture’s by location?
Goodpasture’s does not affect the URT
What histopathologic type of pleuritis may result from PE? What is the diagnostic test of choice for PE?
Fibrinous pleuritis; SPIRAL CT
What marker is useful in Wegener’s? What does it seem to be reacting against?
c-ANCA reacting against Neutrophil Proteinase-3
What are 3 vasculitis-associated hemorrhages in the pulmonary system?
Hypersensitivity angiitis, Wegener Granulomatosis, and SLE
What antibody isotype is present in lung disease caused by Aspergillus fumigatus? Name of disease?
IgE, Allergic Bronchopulmonary Aspergillosis
What kind of emphysema is caused by Coal Worker Pneumoconiosis? Why is this a weird form of emphysema?
Centriacinar emphysema, it is weird bc black lung also has fibrosis, which is atypical for emphysema
What is the clincial triad of Goodpasture Syndrome?
Diffuse pulmonary hemorrhage, glomerulonephritis, and circulating anti-GBM antibodies
What are the two general (major causes) of hypoplasia of the lung?
Oligohydramnios or decreased intrathoracic space
What is Anti-mitochondrial-antibody (AMA) useful for?
Primary Biliary Sclerosis
Which obstructive disease is most likely to result in lung cancer, why?
Chronic bronchitis because in late stages it can lead to squamous metaplasia to deal with the chronic inflammation, this predisposes to CA
What are the 4 general obstructive diseases of lungs? Which are COPD?
Chronic Bronchitis, Emphysema, Asthma, and Bronchiectasis. COPD = Chronic Bronchitis and Emphysema
Where do laryngeal cartilages come from? Airway cartilages?
Laryngeal cartilage is from neural crest (ectoderm) and airway cartilage is from splanchnic mesoderm
What heart defects are associated with pulmonary HTN, why?
Anything that involves a L-> R shunt, most commonly an ASD since it causes volume overload in the right heart
What is the morphology of the deposition of immunoglobulins in Goodpastures?
Linear deposits on basement membranes of glomerulus and alveoli
What are the genetics of Kartagener Syndrome?
Autosomal Recessive
What is seen in long standing pulmonary edema?
Fibrosis of the alveoli
What is the major cause of contraction atelectasis? What is the treatment?
Fibrotic changes of lung or pleura? No Tx.
What is the most likely method of death in a patient with a sudden and large PE?
Acute cor pulmonale
Differentiate the implication of smoking in chronic bronchitis from centroacinar emphysema:
In centroacinar emphysema the issue is that the smoke is inhibiting antitrypsin and allowing elastase to destroy alveoli, in CB the smoke is killing off the cilia and producing inflammation.
What kind of congestion is seen in areas of pulmonary infarction?
PASSSIVE CONGESTION? Pulmonary infarct is seen as ischemic necrosis on background of passive congestion.
What are the early microscopic findings in chronic bronchitis (2)?
Mucus in the airway, hypertrophy of submucosal glands
In which way does the most common cause of atelectasis shift the mediastinum? What is the most common cause of this atelectasis?
Resorption/Obstruction atelectasis shifts mediastinum towards it; most common cause is EXCESSIVE SECRETION because it leads to a blockage
How long does it take to achieve an airless state in obstruction atelectasis and why?
It takes a few hours because the air that is in the aveolus will be absorbed by the blood vessels
Fibrotic changes of lung and/or pleura are the major cause of this type of atelectasis
Restrictive Atelectasis
What is the deadly, unrefractory form of asthma?
Status asthmaticus
What exactly is a Charcot-Leyden crystal?
It is a crystal formed from EOSINOPHIL MEMBRANE PROTEIN, indicating eosinophils are present
What kind of edema is caused by near drowning?
Microvascular injury pulmonary edema
What are 3 REVERSIBLE microscopic signs of PHTN?
Medial hypertrophy, intimal thickening, and intimal hyperplasia
How is Idiopathic Pulmonary Hemodiderosis different than Goodpasture’s?
There are no antibodies in the serum (it is idiopathic) AND there is no renal involvement
What are the CXR findings in pulmonary embolus?
NORMAL, usually–that’s why you do spiral CT
What lung pathology can Fen-Phen cause? Cardiac?
Pulmonary HTN; Phen-Fen valvulitis
What kind of emphysema is caused by A1AT deficiency? Affects what aspect of lungs?
Panacinar emphysema, affects lower lobes
Where is the cricothyroid muscle from? What innervates it?
Pharyngeal arch 4, superior laryngeal nerve
What is the hallmark of ARDS?
Hyaline membranes at the blood-air barrier
What may cause non-bilious vomiting with an air bubble in the stomach?
Tracheoesophageal fistula
What is the hallmark clinical sign of PHTN?
A progressive dyspnea that began with exertion and now occurs at rest–but can see that is not all that differenc from CHF
When does alveolar fibrosis occur in pulmonary edema?
if the edema is long-standing, as in mitral stenosis
What are 3 signs of PHTN seen on CXR? What is the utility of lung biopsy for the Dx?
1) increased cardiothoracic ratio 2) enlarged pulmonary arteries 3) right ventricular hypertrophy; lung biopsy is not helpful for Dx of PHTN
What are 3 things that nearly all etiologies of PHTN result in?
RVH, medial hypertrophy, and pulmonary atheromas
What are two treatment methods for PE?
Thrombolysis or anticoagulation
What are 4 causes of decreased intrathoracic space?
A diaphragmatic hernia (i.e. of Bochdalek/Morgnani), Cystic adenomatoid malformation, lethal form of osteogenesis imperfecta causing chest wall deformity, and chronic effusion such as hydrops fetalis
What is the lining of a foregut cyst? What are they from?
Pseudostratified columnar epithelium; detached part of embryonic foregut
This presents as a nodule with a pleural lining and served by the aorta
Extralobar sequestration
What does amniotic fluid look like histologically? Why?
Like a thumbprint because of the layers of fetal squames
What happened to a lung that weighs less than it should and has less alveoli than projected for gestational age?
Hypoplasia from oligohydramnios or decreased intrathoracic space
What is the major cause of hemodynamic pulmonary edema?
Increased hydrostatic pressure, as in left heart failure
Why is polydramnios an issue in utero? With which type of pulmonary sequestratant is it associated?
Because the excess volume can lead to strangulation; it is associated with EXTRALOBAR pulmonary sequestration
What are 4 methods of prophylaxis for PE?
Early ambulation post-op, IVC filter, compression stockings, and anticoagulation
Differentiate the pleural linings of extralobar and intralobar sequestrants:
Extralobar are outside of the lung but have their own pleural lining, intralobar are within the lung and have the same pleural lining as the lung
Explain why chronic bronchitis and bronchiectasis lead to recurrent pneumonia:
Chronic bronchitis involves loss of cilia and bronchiectasis involves widening of bronchi so the cilia are not as effective at moving stuff up.
Where is the most common origin of a pulmonary embolus? What kind of thrombus may form from an indwelling central catheter?
Most (95%) come from deep leg veins; an indwelling central catheter can cause a right atrial thrombus
What are 3 IRREVERISBLE microscopic signs of PHTN?
Concentric laminar intimal fibrosis, fibrinoid necrosis, and plexiform lesions
A middle aged man presents with sinusitis, hemoptysis and hematuria. What test should you order?
c-ANCA, this is a classic presentation of Wegener’s Granulomatosis
Essentially, how do you define chronic bronchitis?
Persistent cough with copious sputum for greater than 3 months over a period of 2 years
Where do most PE’s occur anatomically? How do you distinguish a PE from a post-mortem clot?
In the lower lobes of lungs; LINES OF ZAHN–indicates that there was organization of the clot over time
What is the most likely etiology of a PE that looks like it has white holes in it histologically?
Fracture of a long bone–this is a fat embolus
Describe the extent of necrosis and granuloma formation in Idiopathic Pulmonary Hemosiderosis:
There is no necrosis/granuloma formation, just capillary congestion with hemorrhage
Why are there more macrophages than neutrophils in Hamman-Rich syndrome?
Because this is ACUTE INTERSTITIAL PNEUMONIA–the lungs are NOT infected, this is a pnuemonia secondary to something somewhere else in the body like a liver laceration leading to hypovolemic or septic shock
What are the 2 general causes of bronchiectasis? End result?
Congenital malformation of bronchial tree, or post-infection? Either way it is a PERMANENT DILATION of the bronchus
Which type of asthma has normal serum IgE, most common cause?
Non-Atopic asthma from infection, usually
What term best describes PHTN w/o underlying parenchymal disease of the lung?
Primary Pulmonary HTN
What are the three types of atelectasis and in which way does the mediastinum shift?
Resorptive/Obstructive atelectasis shifts the mediastinum towards it; Compression atelectasis shifts the mediastinum away; and contraction atelectasis does NOT shift the mediastinum
Though the etiology of of Idiopathic pulmonary hemosiderosis is unknown there is a favorable response to what Tx?
Immunosuppressives
Why doesn?t administration of oxygen help in ARDS?
Because the hallmark of ARDS is hyaline change and hyaline is waxy/thick and creates a diffusion barrier in the blood-air membrane
What isotype is the antibody in Goodpasture’s? What is it actually against?
IgG; Type IV collagen of the basement membrane of glomeruli and lungs
What two systems are compromised in PE? Explain.
Respiratory compromise because the alveoli cannot exchange gas with an occluded vessel; Hemodynamic compromise from the increased resistance from the embolus.
Who are the blue bloaters? Who are the pink puffers?
Blue bloaters = chronic bronchitis and pink puffers = emphysema
What differentiates asthma from COPD, in general?
Reversible bronchospasm
Who gets Curshmann spirals and Charcot-Leyden crystals? What would a Charcot-Leyden crystal in a stool sample indicate?
Asthmatics; it would indicate a parasitic GI infection
What are the treatments of the reversible causes of atelectasis?
Obstructive/resporption can be treated by removing the blockage and compression can be removed by sucking out the intrapleural compression (air, fluid, etc)
Why is pulmonary infarction from a PE rare in young people? What wil you see in them?
Because they usually have adequate bronchial circulation. Infarction really only happens if inadequate circulation, BUT they will have hemorrhage.
What 3 regions are affected by Wegener’s Granulomatosis? What term best describes the inflammation?
Upper Respiratory Tract, Lower Respiratory Tract and Kidneys; Necrotizing GRANULOMATOUS inflammation
When are small emboli in the lungs problematic?
Usually only if there is inadequate bronchial circulation or if there are a ton of them
Which types of emphysema both predominately affect the upper lungs?
Centricacinar/Centrilobular caused by smoking and Distal Acinar/Paraseptal–bullous emphysema
What are c-ANCA and p-ANCA good for diagnostically?
They are commonly elevated in systemic vasculitides
What pressures define PHTN when at rest and exercise?
Greater than 25 mmHg at rest and greater than 30 with exercise
Which obstructive disease is most likely caused by insipissated mucus? What is the cause for this mucus?
Bronchiectasis from cystic fibrosis
A pulmonary infarct with an intense neutrophilic reaction is most likely what?
A septic infarct from a septic embolus
What is the diagnostic test for deep vein thrombosis?
Duplex ultrasonography
What is the hallmark of acute interstitial pneumonia? AKA?
Diffuse damage with ridiculously thickened alveolar walls–lots of macro’s but few neutro’s b/c not infected? HAMMAN-RICH SYNDROME
What is the clinical presentation of of Idiopathic Pulmonary Hemosiderosis?
Intermittent hemoptysis and refractory anemia
What is the main cause of death in Goodpasture’s? What is the Tx?
Uremia from Kidney failure; can Tx with plasma exchange and immunosuppressive Tx
Laryngeal muscles innervated by the recurrent laryngeal nerve are from which pharyngeal arch?
6
What are plexiform lesions? What do they indicate about the severity of the disease? What vessels do they affect?
They are TUFTS of capillaries growing off of a parent artery; the indicate HIGH SEVERITY; they affect small vessels
Trousseau’s sign and protein C/S deficiency are risk factors for what? Why?
Pulmonary embolus (or any thrombotic event, really) because they indicate systemic hypercoagulability
What kind of heart failure will a patient with pulmonary embolism display? Sx?
ACUTE cor pulmonale (right heart failure)–JVD, peripheral edema, pulsatile liver, hepatic ascites (nutmeg liver)
What are you most concerned with as a follow up to organization and dissolution of a PE?
The development of PHTN–> this is because scarring is a part of the organization process which increases resistance.
A tension pneumothorax would shift the mediastinum in which direction?
Toward the healthy side, this is because tension pneumo is a form of compression atelectasis
How is the cause of death different in Idiopathic Pulmonary Hemosiderosis than in Goodpasture’s?
IPH will be a pulmonary-related death whereas Goodpasture’s is usually uremia–a renal issue
What is the most common cause of resorption atelectasis?
Excess Secretion
What kind of pulmonary edema can amphotericin B (amphoterrible) and heroin cause?
Microvascular injury pulmonary edema
What happens to 1) Nearby lung tissue 2) Diaphragm and 3) Chest wall in a resorption atelectasis?
The nearby tissue distends, the diaphragm elevates, and the chest wall flattens
