Week 6 Lecture 2 Flashcards
What are the systolic and diastolic pressures in the pulmonary artery?
Systolic pressure is about 25 mm Hg and diastolic pressure is about 8 mm Hg.
What is the mean pulmonary arterial pressure (mPAP)?
Mean pulmonary arterial pressure is 15 mm Hg.
What is the driving pressure in the pulmonary circulation?
The driving pressure is 10 mm Hg.
What is pulmonary hypertension?
A heterogenous disease involving pathogenic remodeling of the pulmonary vasculature, increasing pulmonary artery pressure and vascular resistance.
What has the mean pulmonary artery pressure (mPAP) threshold for diagnosing pulmonary hypertension been lowered to?
The threshold has been lowered from ≥25 mmHg to >20 mmHg.
What does the classification of pulmonary hypertension include?
It includes various groups based on etiology and hemodynamics.
What histopathological changes occur in mild pulmonary hypertension?
Thickening of the media.
What are the severe histopathological changes in pulmonary hypertension?
Extensive intimal fibrosis and muscle thickening.
What is idiopathic pulmonary arterial hypertension (IPAH)?
A progressive disease leading to right heart failure and early mortality.
What is the gender ratio for idiopathic PAH prevalence?
Women to men by ~3.1-fold.
What is a common clinical symptom of pulmonary arterial hypertension?
Insidious onset of dyspnea.
What is a significant outcome of severe pulmonary hypertension?
Patients die of cor pulmonale.
What are common clinical features of pulmonary edema?
Difficulty breathing (dyspnea), cough producing frothy sputum, rapid heartbeat, anxiety.
What is the definition of pulmonary edema?
An abnormal accumulation of fluid in the interstitial and alveolar spaces of the lung.
What is the most common cause of pulmonary hypertension associated with lung disease?
Chronic obstructive pulmonary disease (COPD).
What are the three components of Virchow’s triad in venous thromboembolism?
Stasis of blood, alterations in the blood coagulation system, abnormalities of the vessel wall.
What is the most common genetic mutation leading to hypercoagulability?
Factor V Leiden.
What are common clinical risk factors for venous thromboembolism?
- Cancer
- Obesity
- Cigarette smoking
- Systemic arterial hypertension
- COPD
- Chronic kidney disease
- Long-haul air travel
- Estrogen-containing contraceptives
- Pregnancy
- Surgery and trauma
- Sedentary lifestyle.
What is the definition of pulmonary embolism?
Thrombi form in large veins and travel to the lungs where they occlude the pulmonary circulation.
What are the types of pulmonary embolism?
- Venous thrombi
- Nonthrombotic emboli: fat, air, and amniotic fluid.
What characterizes a massive pulmonary embolism?
Systemic arterial hypotension and extensive thrombosis affecting at least half of the pulmonary vasculature.
What is chronic thromboembolic pulmonary hypertension (CTEPH)?
Development of pulmonary hypertension after chronic thromboembolic obstruction of the pulmonary arteries.
What is cor pulmonale?
Right ventricular failure due to excessively high pulmonary artery pressures.
What is the pathology of cor pulmonale?
Markedly hypertrophied right ventricle with dilation and loss of normal curvature of the interventricular septum.
What are some causes of cor pulmonale?
- Pulmonary emboli
- Pulmonary vascular disease
- Parenchymal disease.
What characterizes vasculitis?
Inflammation of and damage to blood vessels, leading to compromised vessel lumen and ischemia.
How is vasculitis classified?
By vessel size: small, medium, and large.
Which vasculitides commonly involve lung involvement?
- Wegener’s granulomatosis
- Microscopic polyangiitis
- Churg-Strauss syndrome.
What is the prognosis for untreated Churg-Strauss syndrome?
Poor prognosis, but corticosteroid therapy is almost always effective.
What is granulomatosis with polyangiitis?
Systemic necrotizing vasculitis with granulomatous lesions of the nose, sinuses, lungs, and kidneys.
What are the outcomes associated with pulmonary vascular disorders?
- Pulmonary Arterial Hypertension
- Pulmonary edema
- Pulmonary embolism (acute, chronic)
- Cor pulmonale
- Vasculitis.
Which gender is more affected by Granulomatosis with Polyangiitis (GPA)?
Men are affected more than women, usually in their fifth and sixth decades.
What percentage of patients with GPA are positive for ANCA?
Over 90% of patients with GPA are positive for ANCA.
What is the most prominent pulmonary feature of GPA?
Persistent bilateral pneumonitis with nodular infiltrates that undergo cavitation similar to tuberculosis.
What is sarcoidosis characterized by?
A systemic disorder characterized by noncaseating granulomatous inflammation at sites of disease.
Which organs are most commonly involved in sarcoidosis?
- Lungs
- Intrathoracic lymph nodes
What is the differential diagnosis for sarcoidosis?
- Mycobacterial and fungal infections
- Malignancy
- Environmental agents such as beryllium
What are the contributing factors to the etiology of sarcoidosis?
- Genetic susceptibility
- Environmental exposures
- Infectious or noninfectious environmental agents
What percentage of sarcoidosis cases show familial clustering?
Familial clustering of sarcoidosis has been estimated to involve 10% of cases.
What is the hallmark of the pathology in sarcoidosis?
The granuloma is the pathologic hallmark.
What cells accumulate in the granulomas of sarcoidosis?
- T helper cells
- Activated monocytes
What are the clinical stages of sarcoidosis?
- Stage 0: No pulmonary sarcoidosis
- Stage 1: Granulomas in lymph nodes only
- Stage 2: Granulomas in lymph nodes and lungs
- Stage 3: Granulomas in lungs only
- Stage 4: Pulmonary fibrosis
What is Lofgren’s syndrome characterized by?
- Erythema nodosum
- Hilar adenopathy
- Periarticular arthritis in some cases
What are common pulmonary function test findings in sarcoidosis?
- Restrictive impairment
- Reduction in lung volumes, FVC, and FEV1
- Reduction in diffusing capacity
What are the extrapulmonary manifestations of sarcoidosis?
- Lupus pernio
- Maculopapular lesions on the trunk
What is the relationship of systemic lupus erythematosus to pulmonary issues?
- Pleuritis with or without pleural effusion
- Pulmonary infiltrates
- Life-threatening pulmonary manifestations
What are the complications associated with asbestos exposure?
- Nonmalignant pleural manifestations
- Asbestosis
- Malignant mesothelioma
- Lung cancer
What characterizes asbestosis?
Interstitial pneumonitis and fibrosis resembling other forms of diffuse interstitial fibrosis.
What is silicosis and its main feature?
An occupational pulmonary hazard characterized by free silica (SiO2), or crystalline quartz.
What is the significance of coal workers’ pneumoconiosis (CWP)?
It has enormous social, economic, and medical significance in coal mining industries.
What are the clinical features of coal workers’ pneumoconiosis?
- Chronic cough
- Sputum production
- Dyspnea in advanced cases
What does beryllium disease resemble?
Chronic granulomatous disease resembling sarcoidosis.
What is the latency period for chronic beryllium disease after exposure?
A long time interval or latency of 10 years.
What are the systolic and diastolic pressures in the pulmonary artery?
Systolic pressure: 25 mm Hg, Diastolic pressure: 8 mm Hg.
What is the mean pulmonary arterial pressure compared to the aorta?
Mean pulmonary arterial pressure: 15 mm Hg; Aorta: 93 mm Hg.
How is the driving pressure calculated in the pulmonary artery?
Driving pressure (10 mm Hg) = Mean pressure in pulmonary artery (15 mm Hg) - Pressure in left atrium (5 mm Hg).
What is pulmonary hypertension?
A heterogenous disease involving pathogenic remodeling of the pulmonary vasculature, increasing pulmonary artery pressure and vascular resistance.
What is the adjusted mean pulmonary artery pressure (mPAP) for diagnosing pulmonary hypertension?
mPAP used to diagnose PH has been lowered from ≥25 mmHg to >20 mmHg.
What is considered a mean pressure at rest according to Harrison?
Mean pressure over 25 mm Hg at rest.
What are the histopathological changes seen in late gestation pulmonary arteries?
Pulmonary arteries have thick walls.
What occurs to pulmonary arteries after birth?
The vessels dilate, and the walls become thin.
What is observed in mild pulmonary hypertension?
Thickening of the media.
What characterizes more severe pulmonary hypertension?
Extensive intimal fibrosis and muscle thickening.
What is Idiopathic Pulmonary Arterial Hypertension (IPAH)?
A progressive disease that leads to right heart failure and early mortality.
What is the gender prevalence of IPAH?
Women are affected ~3.1-fold more than men, especially in their 20s and 30s.
What is the prognosis comparison of men and women diagnosed with IPAH?
Men have more severe hemodynamics at diagnosis and a less favorable prognosis compared to women.
What is the effect of elevated right ventricular afterload in PAH?
Right ventricular work must increase to preserve cardiac output.
What are common symptoms of PAH?
Insidious onset of dyspnea, cough with frothy sputum, palpitations, anxiety, and cold clammy skin.
What is pulmonary edema?
An abnormal accumulation of fluid in the interstitial and alveolar spaces of the lung.
What are frequent causes of pulmonary edema?
Various heart and lung diseases.
What is interstitial edema?
Increased lymph flow from the lung and widened lymphatics.
What is alveolar edema?
Fluid moves across the epithelium into the alveoli, preventing ventilation.
What is the clinical picture of pulmonary edema?
Difficulty breathing, orthopnea, cough with blood, rapid heartbeat, and anxiety.
What is the second most common cause of pulmonary hypertension?
Pulmonary hypertension associated with lung disease.
What is venous thromboembolism (VTE)?
Includes deep-venous thrombosis (DVT) and pulmonary embolism (PE).
What are the components of Virchow’s triad?
- Stasis of blood
- Hypercoagulability
- Abnormalities of the vessel wall.
What is the most common autosomal dominant genetic mutation associated with hypercoagulability?
Factor V Leiden.
What are common clinical risk factors for VTE?
- Cancer
- Obesity
- Cigarette smoking
- Systemic arterial hypertension
- COPD
- Chronic kidney disease
- Long-haul air travel
- Estrogen-containing contraceptives
- Pregnancy
- Surgery and trauma
- Sedentary lifestyle.
What is the definition of Pulmonary Embolism?
Thrombi form in large veins and travel to the lungs where they become lodged in and occlude the pulmonary circulation
It is associated with significant morbidity and mortality and can be challenging to diagnose.
What are the types of Pulmonary Embolism?
- Venous thrombi
- Nonthrombotic emboli (fat, air, and amniotic fluid)
What are the similarities between Venous Thromboembolism and Atherothrombosis?
Similar risk factors and similar pathophysiology: inflammation, hypercoagulability, and endothelial injury.
How much more likely are patients who suffer PE to have a future myocardial infarction or stroke?
More than twice as likely.
What characterizes Massive (High-Risk) PE?
Systemic arterial hypotension and extensive thrombosis affecting at least half of the pulmonary vasculature.
What are the clinical features of Massive (High-Risk) PE?
- Dyspnea
- Syncope
- Hypotension
- Cyanosis
What is the prognosis for patients with Low-risk PE?
These patients have an excellent prognosis.
What is CTEPH?
Development of pulmonary hypertension after chronic thromboembolic obstruction of the pulmonary arteries.
What percentage of patients develop CTEPH following a single pulmonary embolic event?
Between 3 and 7%.
What is Cor Pulmonale?
Right ventricular failure due to excessively high pulmonary artery pressures.
What are common causes of Cor Pulmonale?
- Pulmonary emboli
- Pulmonary vascular disease (e.g., collagen vascular disease such as scleroderma)
- Parenchymal disease (e.g., COPD, pulmonary fibrosis)
What is the definition of Vasculitis?
A number of distinct disorders characterized by inflammation of and damage to blood vessels.
How is Vasculitis classified?
By vessel size: small, medium, and large.
What are the primary idiopathic small-vessel vasculitides that involve the lungs?
- Wegener’s granulomatosis
- Microscopic polyangiitis
- Churg-Strauss syndrome
What characterizes Allergic Granulomatosis and Angiitis (Churg-Strauss Syndrome)?
Widespread necrotizing lesions of small and medium-sized arteries with granulomas and eosinophilic infiltrates.
What is the most prominent pulmonary feature of Granulomatosis with Polyangiitis?
Persistent bilateral pneumonitis, with nodular infiltrates that undergo cavitation.
What are the clinical stages of Sarcoidosis?
- Stage 0: No pulmonary sarcoidosis on X-rays
- Stage 1: Granulomas in lymph nodes only
- Stage 2: Granulomas in lymph nodes and lungs
- Stage 3: Granulomas in lungs only
- Stage 4: Pulmonary fibrosis or permanent scarring
What is Lofgren’s Syndrome?
Erythema nodosum and hilar adenopathy with a good prognosis.
What is the typical pulmonary function test result in sarcoidosis?
Restrictive impairment with reduction in lung volumes, FVC, and FEV1.
What is the cause of sarcoidosis?
The cause remains unknown but results from genetic susceptibility and environmental exposures.
What is the hallmark of pathology in sarcoidosis?
Noncaseating granulomatous inflammation.
What is the role of cytokines in sarcoidosis?
Cytokines and other circulating mediators contribute to organ damage and patient symptoms.
What is a common skin manifestation of sarcoidosis?
Maculopapular lesions on the trunk.
What is a life-threatening pulmonary manifestation of Systemic Lupus Erythematosus?
Intra-alveolar hemorrhage.
What is pleuritis?
Inflammation of the pleura, the membrane surrounding the lungs
Pleuritis can cause sharp chest pain and difficulty breathing.
What does ILD stand for?
Interstitial Lung Disease
ILD refers to a group of lung diseases affecting the interstitium.
What is pulmonary hypertension?
Increased blood pressure in the pulmonary arteries
Can lead to heart failure if untreated.
What are occupational lung disorders?
Diseases caused by environmental agents in the workplace
These can include exposure to dust, chemicals, and other hazards.
What factors may interact with occupational and environmental factors?
Smoking and genetic risk
These interactions can increase the risk of developing lung diseases.
What are key inquiries to make regarding occupational exposures?
Specific work practices, contaminants involved, visible dusts, chemical odors, workspace ventilation, use of protective equipment, coworker complaints
These factors help assess the risk of lung disease.
What is asbestos?
A fibrous hydrated magnesium silicate used for insulation due to its fire resistance
Asbestos is known for its durability but poses significant health risks.
What are the types of asbestos exposure?
- Primary exposure: miners and millers
- Secondary exposure: manufacturing plants using asbestos in textiles, friction materials, vinyl tiles, insulation
Different industries have varying levels of risk based on exposure type.
What are complications of asbestos exposure?
- Nonmalignant pleural manifestations
- Asbestosis
- Malignant mesothelioma
- Lung cancer
These complications are serious and can be life-threatening.
What characterizes asbestosis?
Interstitial pneumonitis and fibrosis resembling other forms of diffuse interstitial fibrosis
Early lesions show discrete fibrosis areas in respiratory bronchioles.
What are the signs and symptoms of asbestosis?
- Dyspnea
- Bibasilar rales
- Restrictive ventilatory impairment
Symptoms can worsen over time with continued exposure.
What is silicosis?
A disease caused by exposure to free silica (SiO2) or crystalline quartz
It is one of the oldest known occupational pulmonary hazards.
What is the difference between simple and complicated silicosis?
- Simple silicosis: Small rounded opacities in upper lobes, usually without lung function impairment
- Complicated silicosis: Progressive massive silicosis with obstructive and restrictive impairment
Simple silicosis can develop into complicated silicosis over time.
What is Coal Workers’ Pneumoconiosis (CWP)?
A disease caused by occupational exposure to coal dust
It has significant social, economic, and medical impact.
What is Caplan syndrome?
A condition caused by coal/silica exposure and rheumatoid arthritis
It involves the formation of nodules in the lungs.
What is the pathology of Coal Workers’ Pneumoconiosis?
Coal macule, a focal collection of coal dust in macrophages around respiratory bronchioles
This is a key histopathologic finding in CWP.
What are the clinical features of coal mine dust exposure?
Variable clinical presentations with possible chronic cough and sputum production
Symptoms may not be present in miners with simple pneumoconiosis.
What are the physiological defects associated with CWP?
- Obstructive defects
- Restrictive defects
- Mixed defects
The predominant abnormality depends on individual factors.
What is Beryllium Disease?
A disease associated with industrial exposure to beryllium, with a long latency period
Onset can occur up to 10 years after initial exposure.
What are the types of Beryllium Disease?
- Acute pneumonitis
- Chronic granulomatous disease resembling sarcoidosis
Chronic Beryllium Disease can lead to significant lung damage.
