5.Obstructive lung diseases-Restrictive lung disease-Obstructive vs. restrictive lung disease-Hypersensitivity pneumonitis-Pneumoconioses Flashcards
What is the definition of obstructive lung disease? How does it affect lung volumes?
Obstruction to flow leading to air trapping & the collapse of airways at high volumes; RV increases due to air trapping, & FVC decreases
Does residual volume increase or decrease in obstructive lung disease? How about functional vital capacity?
RV increases; FVC decreases
What is the hallmark pulmonary function test finding in patients with obstructive lung disease?
Decreased ratio of FEV1 to FVC (the decrease in FEV1 > FVC)
A patient with chronic, hypoxic vasoconstriction can have what cardiac manifestation?
Cor pulmonale
List four types of obstructive lung disease.
Chronic bronchitis, emphysema, asthma, and bronchiectasis
A patient has a productive cough for 5 months over the course of 3 years. What findings do you expect on pulmonary function tests?
PFTs show a decreased ratio of FEV1 to FVC (the patient has classic chronic bronchitis)
A man has a productive cough for 5 months over 2 years with wheezing, crackles, cyanosis. What histologic changes are seen on lung biopsy?
Hyperplasia of the mucus-secreting glands in the bronchi (the patient has chronic bronchitis)
The mucus gland hyperplasia seen in chronic bronchitis can be quantified using the ____, which tends to be greater than what value (in %)?
Reid index (thickness of gland layer/total thickness of bronchial wall); >50%
How is the Reid index calculated?
Reid index = gland layer thickness/total bronchial wall thickness
A patient with chronic bronchitis presents to clinic. What clinical findings are auscultated in the lungs of this patient?
Usually wheezing and crackles
What visible skin finding may be noted in patients with chronic bronchitis?
Cyanosis (early-onset hypoxemia from shunting)
A woman has 6 months of productive cough in 3 years, wheezing, crackles, and cyanosis. What other findings would you expect in this patient?
• Expect late-onset dyspnea, secondary polycythemia, and hypercapnia (this patient has chronic bronchitis, as she is a “blue bloater”)
In emphysemic lungs, there is a(n) ____ (decrease/increase) in recoil and a(n) ____ (decrease/increase) in compliance.
Decrease, increase
Name the two types of emphysema.
Centriacinar and panacinar
A patient is diagnosed with α1-antitrypsin deficiency. What pattern of alveolar damage is this associated with?
Panacinar (this is emphysema)
In emphysema, the loss of elastic fibers and increased lung compliance result from increased activity of which enzyme?
Elastase
Individuals with emphysema tend to exhale through pursed lips to increase ____ and prevent ____ during expiration.
Airway pressure, airway collapse
In patients with asthma, there is hyperresponsiveness of what lung segment?
The bronchi
An important feature of the bronchoconstriction in asthma is that it is ____.
Reversible
A man with cough, tachypnea, and wheezing has a drop in BP >10 mmHg on inspiration. What pathologic lung findings do you expect on biopsy?
Curschmann spirals, smooth muscle hypertrophy, Charcot-Leyden crystals (this is asthma, which can exhibit pulsus paradoxus when severe)
What are Charcot-Leyden crystals?
Formed after the breakdown of eosinophils in sputum, they are eosinophilic, hexagonal, double-pointed needle-shaped crystals in asthmatics
Name some triggers for bronchial hyperresponsiveness in asthmatics
Allergens, viral URIs, stress
A child with cough, wheezing, dyspnea, and tachypnea has pulsus paradoxus on exam. What is a test for his condition?
Test with methacholine challenge (the patient has asthma)
What finding is commonly noted on pulmonary function tests of patients with asthma?
Decreased inspiratory:expiratory ratio
You measure the blood pressure of a patient having a severe asthma attack. What phenomenon might you observe with repeat measurements?
Pulsus paradoxus
____ is a chronic necrotizing infection of the bronchi.
Bronchiectasis
In bronchiectasis, chronic necrotizing infection of the bronchi leads to ____ (permanent/reversible) dilation of airways.
Permanent
A patient with bronchiectasis feels ill and has a productive cough. His sputum is most likely to consist of what?
Blood and purulence
Recurrent infections, bronchial obstruction, and poor ciliary motility may lead to what lung manifestation?
Bronchiectasis
Which two genetic diseases are associated with bronchiectasis?
Cystic fibrosis and Kartagener syndrome
A patient has Kartagener syndrome leading to permanently dilated airways. Which fungal pulmonary infection is he prone to developing?
Allergic bronchopulmonary aspergillosis (he likely has bronchiectasis)
What two lung volumes are typically decreased in patients with restrictive lung disease?
Functional vital capacity and total lung capacity
Patients with restrictive lung disease typically have a FEV1/FVC ratio within what range?
≥80%
What are the two general types of restrictive lung disease?
Poor breathing mechanics and interstitial lung diseases
Extrapulmonary causes of restrictive lung disease are generally the result of what?
Poor breathing mechanics from muscular (e.g., polio, myasthenia gravis) or structural (e.g., scoliosis, morbid obesity) dysfunction
Mechanical restrictive lung diseases have a(n) ___ A-a gradient. Interstitial lung diseases have a(n) ___ A-a gradient.
Normal; increased
What infectious disease can cause poor muscular effort and lead to extrapulmonary restrictive lung disease and peripheral hypoventilation?
Polio
What disease of the neuromuscular junction can cause poor muscular effort and thereby lead to extrapulmonary restrictive lung disease?
Myasthenia gravis
A 32-y/o woman with neuromuscular disease, ptosis, diplopia, and muscle weakness at the day’s end may have what pulmonary manifestation?
Restrictive lung disease from poor muscular effort (the patient likely has myasthenia gravis)
Other than scoliosis and muscle diseases, what condition can lead to extrapulmonary restrictive lung disease?
Morbid obesity
Pulmonary causes of restrictive lung disease are generally the result of what category of diseases?
Interstitial lung diseases
Restrictive lung disease due to acute respiratory distress syndrome (ARDS) would have ____ (increased/decreased) diffusing capacity.
Decreased
A premature infant is diagnosed with hyaline membrane lung disease. What type of restrictive is this?
Interstitial type (this is neonatal respiratory distress syndrome [NRDS], also known as hyaline membrane disease)
What category of interstitial lung diseases has a clear association with an environmental exposure?
Pneumoconioses (e.g., anthracosis, silicosis, asbestosis)
A patient has bilateral hilar lymphadenopathy, noncaseating granulomas, and hypercalcemia. What interstitial lung disease does she have?
Sarcoidosis
A patient has an autoimmune disease with hemoptysis and hematuria with renal failure. What kind of lung disease does this patient have?
Restrictive lung disease (Goodpasture syndrome)
What granulomatous small-to-medium vessel vasculitis causes both glomerulonephritis and pulmonary dysfunction?
Granulomatosis with polyangiitis (Wegener)
What disease can cause restrictive lung disease due to eosinophilic granulomas in the lungs?
Langerhans cell histiocytosis
A patient has a cough and dyspnea at work that resolves when he leaves. What restrictive pulmonary process is likely causing his symptoms?
Hypersensitivity pneumonitis
Name four drugs that can cause interstitial lung disease.
Bleomycin, busulfan, methotrexate, and amiodarone
A patient has progressive dyspnea. Serum ACE and Ca2+ levels are elevated. What will a CXR show?
Bilateral hilar lymphadenopathy (this is likely sarcoidosis)
In a normal, well-functioning lung, what percent does the FEV1:FVC ratio equal?
80%
• In ____ (obstructive/restrictive) lung disease, FEV1 is more reduced than FVC.
Obstructive
In ____ (obstructive/restrictive) lung disease, the FEV1:FVC ratio and lung volumes are increased.
Obstructive
In ____ (obstructive/restrictive) pulmonary disease, the FEV1:FVC ratio and lung volumes are decreased.
Restrictive
A COPD patient has PFTs performed. What would you expect his TLC, FRC, and RV to be compared to normal?
In obstructive lung disease, TLC, FRC, and RV are all increased
A patient with silicosis has PFTs performed. What would you expect his FEV1 and FVC to be compared to normal?
In restrictive lung disease, both FEV1 and FVC are reduced
A bird keeper develops dyspnea, cough, chest tightness, and a headache. What is the pathophysiology of his disorder?
A mixed III/IV hypersensitivity to environmental antigens (common in farmers and those around birds) (this is hypersensitivity pneumonitis)
Silicosis, coal workers’ pneumoconiosis, and asbestosis are associated with increased risk of what two other diseases?
Cor pulmonale and Caplan syndrome (the combination of pneumoconiosis and rheumatoid arthritis)
What occupations are associated with exposure to asbestos?
Shipbuilding, roofing, and plumbing
Asbestosis mainly affects the ____(lower/upper) lung lobe(s), whereas silica and coal workers’ lung affects the ____(lower/upper) lobe(s).
Lower, upper
A patient who works in an aerospace company is at risk for which type of pneumoconiosis?
Berylliosis, as working in the aerospace or manufacturing industries increases one’s risk for beryllium exposure
A patient presents with shortness of breath and cough. He works for a local aerospace manufacturing plant. Treatment?
Steroids, as granulomas arise in berylliosis that may respond to this treatment
Which lobes of the lungs are affected in a patient with berylliosis?
Upper lobes
Explain the pathogenesis of coal workers’ pneumoconiosis (also known as black lung disease).
Prolonged exposure to coal dust results in carbon within macrophages, which leads to inflammation and fibrosis in the lungs
Which lobes of the lungs are most affected by coal miners’ disease?
Upper lobes
A patient is diagnosed with anthracosis. How does this differ from coal workers’ pneumoconiosis
Anthracosis is the result of exposure to sooty air in the city versus coal dust in coal workers (it is asymptomatic)
Patients with silicosis typically work in which three fields?
Foundries, sandblasting, mining
A foundry worker has dyspnea and cough. Chest X-ray shows calcification of his hilar lymph nodes. What cell type is responsible?
Macrophages respond to silica exposure by releasing fibrogenic factors, leading to fibrosis of the lungs (the patient has silicosis)
How does silicosis increase the risk of susceptibility to tuberculosis?
Silica impairs macrophage phagolysosomes, affecting their ability to effectively kill microbes
A sandblaster has egg-shell calcification on chest X-ray. What disorders is this patient at increased risk of developing?
This patient has silicosis, increasing risk for TB and bronchogenic carcinoma
A foundry worker has dyspnea and cough. Chest X-ray shows hilar lymph node calcifications. He is at increased risk of what cancer type?
Bronchogenic carcinoma
Which lobes of the lung are most affected by silicosis?
Upper lobes
What is a mnemonic that helps you remember which pneumoconiosis affects the upper and lower lobes of the lungs?
Asbestos = from roof (insulation) but affects the base (lower lobes), silica and coal = from base (earth) but affect the roof (upper lobes)
