Flashcards in Path x2 Deck (127):
What is bronchiectasis?
Permanent dilation of the bronchi and bronchioles, caused by destruction of muscle and elastin tissue
Is bronchiectasis reversible?
What are the two requisite conditions for bronchiectasis?
Obstruction and chronic, persistent infections
What is the histological change with bronchiectasis?
FIbrosis of the bronchioles, holding them open
What are the three common obstructive causes of bronchiectasis?
What are the two congenital conditions that cause bronchiectasis?
What type of pneumonia causes bronchiectasis? What organism?
Staph Aureus, klebsiella, or TB
What chromosome is responsible in CF?
Why is there thick mucus with CF?
NaCl is pulled inward, opposite of sweat glands
What is the defect with Kartagener syndrome?
Structural defect in dynein of cilia
Is Kartagener syndrome AR or AD?
What are the histological findings of the cilia with Kartagener syndrome?
Loss of the radial spokes
What are the ssx of bronchiectasis? (4)
Foul smelling sputum
What are the complications that can arise from bronchiectasis? (4)
What is the major, basic issue with restrictive diseases?
Fibrosis of the lungs causes problems getting air in
What type of lung disease is kyphoscoliosis?
What are the two external causes of restrictive lung diseases?
Deformed chest wall
Pleural space filled with stuff
What are the hallmarks of chronic, diffuse interstitial diseases? What is the consequence of this?
Reduced compliance d/t inflammation and fibrosis.
What happens to TLC with restrictive lung diseases? FEV1? FEV1/FVC?
What is the typical presentation of interstitial lung diseases? Lung sounds?
What are the x-ray findings of interstitial lung disease?
Diffuse, bilateral infiltrative lesions, or ground glass shadows
What are the complications of interstitial lung diseases?
What is the end stage result of ILDs?
What is acute lung injury?
Capillary damage causing non-cardiogenic pulmonary edema
What is the clinical presentation of acute lung injury?
Abrupt onset of significant hypoxemia and pulmonary infiltrates
What is ARDS?
Acute respiratory distress syndrome causing diffuse alveolar capillary damage
What are this histological manifestations of ARDs and acute lung injury?
Diffuse alveolar damage
What is the severe sequelae of ARDS?
Multisystem organ failure
What is the most common cause of noncardiogenic pulmonary edema?
What are the three direct injures (from outside) that cause ARDS?
What are the four major indirect (from inside) causes of ARDS?
What are the CXR findings of ARDS?
bilateral infiltrates on CXR
What happens to pulmonary capillary wedge pressure with ARDS?
Is less than 18 mmHg
What happens to PaO2/FiO2 with ALI?
Less than 300
What happens to PaO2/FiO2 with ARDS?
Less than 200
What is the pathogenesis of ARDS?
Uncontrolled activation of acute inflammatory system, leading to an increased vascular permeability and alveolar thickening
What happens to diffusion capacity with ARDS?
What happens to the surfactant with ARDS?
What causes the inflammation in endothelial cells with ARDS?
Complement and TNF-alpha
What causes the destruction of the alveolar capillaries with ARDS?
What is the primary effect of IL-8?
Potent PMN chemoattractant
What is the cause of IRDS?
Deficiency in pulmonary surfactant causes an increase in vascular permeability and alveolar flooding
What is the role of NO with ARDS?
Dilation of the pulmonary vasculature decreases PA pressure and resistance
What is the mortality rate with ARDS?
What are the gross characteristics of the lungs with ARDS?
Heavy, thick, red, firm
What are the histological characteristics of ARDS? (2)
Interstitial and alveolar edema
Transudate or exudate with ARDS?
What are the three phases of ARDS, and when do they occur?
-Acute exudative: 0-7 days
-Proliferative phase 1-3 weeks
-fibrotic/healing 3-4 weeks
What happens in the fibrotic stage of ARDS? (what two cell types are activated)
Fibroblastic proliferation and type II pneumocyte hyperplasia
What is the general progression of ARDS?
1. injury = edema
2. Alveoli collapse, type II pneumocytes increase
Do patients with ARDS always have fibrosis if they recover?
What is TRALI?
Anti-HLA or HNA antibodies cause lung collapse
What are the four major categories of ILD?
What are the three major fibrosing ILDs?
-Usual interstitial pneumonia (UIP)
-Non-specific interstitial pneumonia (NSIP)
-Cryptogenic organizing pneumonia (COP)
What are the two major granulomatous ILDs?
What is pneumoconiosis?
Inorganic material breathed in
What are the two smoking related ILDs?
What is the initial finding of diffuse interstitial disease?
Alveolitis with Leukocyte accumulation
What is the final stage of diffuse interstitial lung diseases?
Fibrotic lung (honeycomb lung)
What is the role of M1 macrophages? M2?
M1 = Inflammation
M2 = healing
What is the major cell type that is implicated with diffuse interstitial disease?
What is idiopathic pulmonary fibrosis?
Pulmonary disorder of unknown etiology characterized by diffuse interstitial fibrosis
What is the histological pattern of idiopathic pulmonary fibrosis?
Usual interstitial pneumonia (UIP)
What is the term for the interstitial fibrosis with idiopathic pulmonary fibrosis?
Cryptogenic fibrosing alveolitis
What is the clinical course of idiopathic pulmonary fibrosis?
slow, Insidious onset of SOB with non-productive cough
What are the complications of late IPF?
How do you diagnose IPF?
Diagnosis of exclusion
What is the prognosis of IPF? What is the treatment?
3 year survival
Lung transplant (NOT steroids)
What is the current theory of IPF?
Repeated cycles of epithelial activation/injury by some agent causes fibrosis
What is the major cell type that is upregulated with IPF? Cytokines?
What is the hallmark histological finding of IPF? What causes this?
What is the main fibrosing component of IPF? What does this do? (3)
-Activates fibroblasts and myofibroblasts
What is the effect of TGF-beta1 on fibroblast caveolin in IPF? What does this cause?
TGF inhibits it
Caveolin can no longer inhibit deposition of collagen
What are the early findings of IPF? (2)
Alveolitis with leukocyte infiltration
What is the hallmark of UIP?
Patchy, interstitial fibrosis
What is the "temporal heterogeneity" seen with IPF?
Over time, lungs become more collagenous and less cellular, but occurring at different rates in different places
What are the late findings of IPF?
Dense fibrosis and collapse of alveolar wall
What causes the honeycomb lung in the end stages of lung diseases?
Dense fibrosis and collapse of the alveolar walls, leading to restructuring of airspaces and obliteration of small airways
What is the consequence of IPF?
What is the only form of ILD does not respond to steroids, and requires a transplant?
True or false: aspiration can lead to honeycomb lung
What are the diseases that can lead to honeycomb lung?
Interstitial granulomatous disease
What are the collagen vascular disease that can cause restrictive lung disease?
What restrictive lung disease can be caused by scleroderma?
What is the main determinant of the pathogenesis of pneumoconioses?
Solubility and size
What does pneumoconiosis lead to?
Acute Lung Injury
What happens to larger particles in pneumoconioses?
Resist dissolution, leading to fibrosis
What is the physicochemical reactivity of the particles in lungs?
Direct tissue damage from releasing free radicals and other chemical groups
What is the key endogenous factor that determines the prognosis with pneumoconioses?
Capacity of inhaled dusts to stimulate fibrosis
What are the three main mediators release by macrophages that cause damage in pneumoconioses?
What are the initial changes with coal exposure? What about for long term?
Emphysema progressing to fibrosis
What is the key characteristic of asbestos?
What is anthracosis?
Benign buildup of carbon in macrophages d/t pollution
What is simple coal workers' pneumoconiosis (CWP)?
1-2 mm Nodules with collagen fibrils, but minimal lung dysfunction.
What is complicated CWP? What are the sequelae of this?
Progressive/massive fibrosis that leads to compromised lung function
Cor pulmonale from fibrosis and pHTN
Is there an increased risk for cancer or TB with CWP?
What is the eventual consequence of CWP?
Pulmonary massive fibrosis (PMF)
What is the "black lung disease"?
Disabling respiratory insufficiency d/t massive pulmonary fibrosis
What is Caplan syndrome? What does this lead to?
Coexistence of rheumatoid arthritis with a pneumoconiosis. Leads to the development of distinctive nodular pulmonary edema
What is the most prevalent occupational disease in the world?
What is the progression of silicosis?
Slowly progressive, nodular fibrosis
What are the ssx of silicosis?
Progressive SOB and cyanosis
What is the pathogenic part of silicosis?
What major cytokine is released when macrophages are exposed to the quartz in silicosis?
Are patients with silicosis more susceptible to cancer and/or TB?
Yes, to both
What are the gross characteristics of silicosis?
Concentrically arranged collagenous nodules that begin as small lesions in the upper lungs, but grow
What are the histological findings of silicosis? What technique can be used to highlight this?
Hyalinized whorls of collagen with scant inflammation
What are the serpentines seen with asbestos? How about amphiboles? Which is more pathogenic and is the one associated with mesothelioma?
Serpentines = Flexible curved rods
*Amphiboles* = Straight rods
Asbestos was classically seen in whom?
Construction yard or shipyard workers
Which type of pneumoconiosis causes pleural plaques and this pleural effusions?
Which is more of an issue with asbestosis: mesothelioma or lung cancer?
Lung cancer develops much sooner
What is the histological pattern seen with asbestos? What else is seen?
Fibrosis and asbestos bodies
True or false: asbestos increases the risk for cancer systemically
What are the characteristics of the asbestos fibers that break through the interstitium to cause diffuse interstitial pulmonary fibrosis?
What are asbestos body?
Asbestos fibers absorbed by macrophages and coated by hemosiderin
What is the general morphology of asbestosis? (what structures are affected/how does it progress)
DIffuse pulmonary fibrosis that begins around respiratory bronchioles and progressives to involve alveoli (honeycomb lung)
Which generally causes upper lobe fibrosis, and which causes lower lobe fibrosis: asbestosis, silicosis
Silicosis = upper lobe
Asbestosis = lower lobg
What is the increase in lung cancer development with asbestos exposure? What about with smoking? What abous with mesotheliomas?
5x with exposure
55x with smoking
1000x with mesothelioma
What causes the 1000x increase in lung CA with mesothelioma?
ROS generation with asbestos fibers
What is the clinical course of asbestosis?
-DOE, later at rest.
-Heart and respiratory failure
What are the drugs that cause lung fibrosis? (2)
What is the use of bleomycin?
What is the use of methotrexate?
What are the drugs that cause hypersensitivity pneumonitis? (2)
What are the two drugs that cause bronchospasm?
What is acute radiation pneumonitis?
1-6 months post radiation causes an inflammatory response, and can cause pleural effusions