Restrictive Lung disease Flashcards
Question 1: What is the primary consequence of Restrictive Lung Disease on the lungs’ air-holding capacity?
Answer 1:
- Restrictive Lung Disease results in a decrease in the total volume of air that the lungs can hold
- due to decreased lung elasticity or issues related to chest wall expansion during inhalation.
Question 2: Describe the role of alveolar macrophages in the pathophysiology of Restrictive Lung Diseases.
Answer 2:
- Alveolar macrophages are activated when exposed to triggers entering the respiratory system.
- They produce cytokines, which activate the immune system and stimulate neutrophils and T-cells, propagating immune response worsening.
Question 3: How does repeated lung injury lead to fibrosis in Restrictive Lung Diseases?
Answer 3:
- Repeated lung injury triggers fibroblasts to produce fibrous tissue,
- leading to its deposition in the lung parenchyma, resulting in fibrosis.
Question 4: What is the term used to describe fibrosis occurring in various locations within the lung tissue?
Answer 4: Fibrosis can occur in different lung locations, leading to the term Diffused parenchymal lung disease.”
Question 5: In what way does fibrosis impact lung compliance and inflatability?
Answer 5:
- Fibrosis significantly reduces lung compliance, resulting in a decrease in the lung’s ability to inhale as much volume of air as normal lungs.
Question 6: Name and explain the two categories of Restrictive Lung Diseases based on the origin of the disorder.
Answer 6:
- Intrinsic Pulmonary Disorders (fibrosis within the lung tissue) and #
- Extrinsic Pulmonary Disorders (issues not originating from lung tissues).
Question 7: What are the three types of disorders that fall under Extrinsic Pulmonary Disorders, and how do they affect lung expansion?
Answer 7:
(1) Pleural Cavity Disorders (pleural effusion and pneumothorax),
(2) Neuromuscular Disorders (muscle and nerve impairments), and
(3) Chest Wall Disorders (bone, joint, and ligament issues). These disorders can prevent proper lung expansion.
Question 8: How do neuromuscular disorders like Myasthenia Gravis and Guillain Barre Syndrome impact lung expansion?
Answer 8:
Neuromuscular disorders affect lung expansion by impairing the muscles or nerves that supply muscles responsible for lifting the ribs and expanding the chest and lungs.
- Myasthenia Gravis involves inhibition at neuromuscular junctions due to antibody attack, while
- Guillain Barre Syndrome causes muscle weakness due to nerve damage.
Question 9: What is the effect of obesity on chest wall expansion in relation to Restrictive Lung Diseases?
Answer 9:
Obesity prevents proper chest wall expansion due to the presence of a fatty layer that limits the chest wall’s ability to expand.
Question 10: How do scoliosis and ankylosing spondylitis impact chest wall movement and expansion?
Answer 10:
- Scoliosis impedes rib movement, while
- ankylosing spondylitis involves inflammation in joints,
- both of which can lead to restricted chest wall movement and expansion.
Question 1: Describe the characteristics of granulomatous restrictive lung diseases and how they restrict lung expansion.
Answer 1:
- the deposition of clumps of macrophages, lymphocytes, giant cells, and fibrous tissues in the lung parenchyma, leading to restricted lung expansion.
- These diseases can be caseating (e.g., tuberculosis) or non-caseating.
Question 2: Name two examples of granulomatous restrictive lung diseases and their respective epidemiological features.
Answer 2:
(a) Sarcoidosis: Common in young African-American females, aged 20-30s.
(b) Hypersensitivity Pneumonitis: Common in farmers and triggered by organic dusts and proteins, such as hay, barley, and pigeon droppings.
Question 3: What is the underlying mechanism of pneumoconiosis in causing restrictive lung disease?
Answer 3: Pneumoconiosis involves exposure to triggers that induce an immune response, leading to fibrosis of the lung parenchyma.
Question 4: List three examples of occupational exposures that can lead to silicosis.
Answer 4:
(a) Sandblasting,
(b) Mines, and
(c) Foundries can expose individuals to triggers that cause silicosis.
Question 5: Name two occupations that could lead to asbestosis and describe the triggers.
Answer 5:
(a) Shipbuilding and
(b) Plumbing are occupations that can lead to asbestosis.
- Asbestos exposure is common in these occupations due to its use in building materials.
Question 6: Provide an example of a disease associated with exposure to beryllium and list some industries where such exposure could occur.
Answer 6:
- Berylliosis can occur due to exposure to beryllium and is commonly associated with industries like aerospace, electronics, ceramics, and manufacturing.
Question 7: Name a type of pneumoconiosis that is linked to coal mining and provide an example of another substance besides coal that could trigger pneumoconiosis.
Answer 7:
(a) Co-Workers Pneumoconiosis (CWP) is linked to coal mines.
(b) Carbon mines are another example of an occupation that can lead to pneumoconiosis.
Question 8: Mention four drugs that can cause fibrosis as a fatal side effect.
Answer 8:
(a) Methotrexate,
(b) Amiodarone,
(c) Nitrofurantoin, and
(d) Bleomycin are drugs known to cause fibrosis as a serious side effect.
*
Question 9: Explain the concept of hypersensitivity pneumonitis and provide examples of triggers.
Answer 9:
- Hypersensitivity pneumonitis is triggered by exposure to organic dusts and proteins.
- Examples include hay, barley, and pigeon droppings, which can induce a granulomatous type of response in the lung.
Question 1: How do collagen vascular diseases contribute to restrictive lung diseases, and what are two possible outcomes of immune responses in these diseases?
Answer 1:
* Collagen vascular diseases, which are immune disorders, can lead to the production of autoantibodies.
- These autoantibodies can either destroy lung tissue directly or cause vasculitis.
- Both outcomes can ultimately result in lung injury and fibrosis.
Question 10: How do granulomatous restrictive lung diseases differ from pneumoconiosis in terms of their underlying mechanisms?
Answer 10:
- Granulomatous restrictive lung diseases involve the deposition of immune cells and fibrous tissue, while pneumoconiosis is caused by exposure to triggers inducing immune responses and fibrosis.
Question 2: Name three examples of collagen vascular diseases that can lead to restrictive lung diseases and describe the specific autoantibodies involved in each case.
Answer 2:
(a) Scleroderma involves antibodies like Anti-SCL 70 and Anti-centromere.
(b) GPA (Granulomatosis polyangiitis) is associated with C-ANCA autoantibodies.
(c) EPA (Eosinophilic polyangiitis) is linked to P-ANCA autoantibodies.
Question 3: How does GPA (Granulomatosis polyangiitis) impact the lungs, and what type of blood vessels are affected?
Answer 3:
- GPA, also known as Granulomatosis polyangiitis or Wagner’s granulomatosis, involves the auto-antibodies C-ANCA.
- It attacks the lung capillaries, leading to lung injury and potential fibrosis.
Question 4: Describe the impact of EPA (Eosinophilic polyangiitis) on the lungs and the type of autoantibodies involved.
Answer 4:
- EPA, also known as Eosinophilic polyangiitis or Churg-Strauss, involves the auto-antibodies P-ANCA.
- It attacks the actual lung tissue, potentially causing fibrosis.
Question 5: How does Good Pasture Syndrome affect the lungs, and what specific autoantibodies are involved?
Answer 5:
- Good Pasture Syndrome involves the auto-antibodies Anti-GBM.
- These antibodies attack the basement membrane around the respiratory membrane, leading to inflammation, lung injury, and eventual fibrosis.
Question 6: In the context of collagen vascular diseases, what is the significance of autoantibodies Anti-SCL 70 and Anti-centromere?
Answer 6:
In collagen vascular diseases like Scleroderma, the presence of autoantibodies such as Anti-SCL 70 and Anti-centromere is significant because they contribute to lung injury and fibrosis.
Question 7: What specific component does Good Pasture Syndrome’s autoantibodies attack, and how does this lead to lung-related issues?
Answer 7:
- attack the basement membrane around the respiratory membrane.
- This leads to inflammation, lung injury, and eventually fibrosis due to the disruption of the membrane’s integrity.
Question 8: Differentiate between GPA and EPA in terms of the type of blood vessels they affect and the autoantibodies involved.
Answer 8:
- GPA targets lung capillaries and involves C-ANCA autoantibodies.
- EPA targets the actual lung tissue and involves P-ANCA autoantibodies.
Question 9: How do collagen vascular diseases exemplify the link between immune responses and restrictive lung diseases?
Answer 9:
Collagen vascular diseases demonstrate how immune responses, in the form of autoantibodies, can trigger lung injury and fibrosis, leading to restrictive lung diseases.
Question 10: Provide a brief overview of the connection between autoantibodies and lung fibrosis in collagen vascular diseases.
Answer 10:
- In collagen vascular diseases, autoantibodies can either destroy lung tissue directly or cause vasculitis, both of which contribute to lung injury.
- This lung injury can lead to fibrosis, which is a common outcome in these diseases.
Question 1: What distinguishes idiopathic restrictive lung disease from other types of restrictive lung diseases, and why is its mechanism of fibrosis uncertain?
Answer 1:
Idiopathic restrictive lung disease is characterized by uncertain or hypothetical reasons for causing fibrosis. Its mechanism of fibrosis is not clearly understood, hence the term “idiopathic.”
1.
Question 2: What is the common factor leading to a diagnosis of idiopathic restrictive lung disease?
Answer 2:
Smoking is one of the factors that may contribute to idiopathic restrictive lung disease, and its diagnosis often includes a history of smoking.
Question 3: Name the classic clinical features associated with restrictive lung diseases.
Answer 3:
(a) Dyspnea (shortness of breath),
(b) Dry Cough (unproductive cough),
(c) Basilar Crackles (auscultation of Velcro-like sound at the base of the lungs),
(d) Nail Clubbing (due to chronic low blood oxygen levels), and
(e) Cor Pulmonale (right heart failure due to underlying lung disease).
Question 4: Explain the mechanism behind the presence of basilar crackles in restrictive lung diseases.
Answer 4:
- Basilar crackles are caused by the popping up of bronchioles and alveoli during inspiration.
- This popping sound occurs because these structures had collapsed due to fibrosis.
Question 5: How does chronic low blood oxygen levels contribute to nail clubbing?
Answer 5:
Chronic low blood oxygen levels stimulate the development of nail clubbing, a condition characterized by bulbous enlargement of the fingertips, as a response to long-term oxygen deprivation.
Question 6: Define “cor pulmonale” and explain how restrictive lung diseases can lead to its development.
Answer 6:
- Cor pulmonale refers to right heart failure due to underlying lung disease.
- In restrictive lung diseases, the respiratory membrane’s impairment leads to uneven blood distribution in the lungs, causing pulmonary vessels to send blood to well-ventilated alveoli.
- However, this can lead to pulmonary artery vasoconstriction and eventually contribute to cor pulmonale.
Question 7: What are some additional symptoms associated with cor pulmonale resulting from restrictive lung diseases?
Answer 7:
Cor pulmonale due to restrictive lung diseases can lead to symptoms such as Jugular Venous Distention (JVD), hepatomegaly, ascites, and pedal edema.
Question 8: How does the extensive fibrosis in restrictive lung diseases affect gas exchange and blood gases?
Answer 8:
- Extensive fibrosis increases the distance that oxygen has to diffuse through into the bloodstream, resulting in hypoxemia (low blood oxygen levels).
- Additionally, the impaired diffusion of carbon dioxide leads to its incomplete diffusion, causing hypercapnia (increased carbon dioxide levels).
Question 9: What are three common symptoms associated with restrictive lung diseases due to the increased fibrosis and impaired gas exchange?
Answer 9:
The three common symptoms are hypoxemia (low blood oxygen levels), tachypnea (rapid breathing), and hypercapnia (increased carbon dioxide levels).
Question 10: What is the significance of nail clubbing in the context of restrictive lung diseases?
Answer 10: Nail clubbing is a physical sign that indicates chronic low blood oxygen levels, which are often seen in individuals with restrictive lung diseases.
Question 1: What are the specific features associated with sarcoidosis and how do they present?
Answer 1:
(a) bilateral hilar lymphadenopathy visible on X-ray,
(b) lupus pernio with a violaceous rash on the face,
(c) erythema nodosum, nodules on the lower extremities,
(d) anterior uveitis with ocular hyperemia and pain,
(e) nephrolithiasis (renal stones) due to excess alpha-hydroxylase activity, and
(f) facial palsy resulting from granuloma deposition along CN 7, leading to facial weakness.
Question 2: How does sarcoidosis contribute to nephrolithiasis and hypercalciuria?
Answer 2:
- Sarcoidosis-associated granulomas can stimulate macrophages to produce excess alpha-hydroxylase.
- This enzyme converts inactive Vitamin D to its active form, leading to increased calcium absorption and hypercalciuria, which can contribute to nephrolithiasis (renal stones).
Question 3: Explain how facial palsy and AV node block can be associated with sarcoidosis.
Answer 3:
- Granulomas in sarcoidosis can deposit along the length of CN 7 (facial nerve), causing facial palsy.
- Additionally, they can also deposit in the AV node and bundle branches of the heart’s conduction system, leading to AV node block and even restrictive cardiomyopathy.
Question 4: Describe the key features of hypersensitivity pneumonitis and its rapid onset and resolution.
Answer 4:
- Hypersensitivity pneumonitis develops within hours of exposure to the triggering substance and resolves within hours of exposure removal.
Question 5: What is the significant risk factor for tuberculosis associated with silicosis?
Answer 5:
- Silicosis is associated with an increased risk of tuberculosis.
- The presence of silica prevents the formation of phagolysosomes, preventing the breakdown of bacteria and increasing susceptibility to tuberculosis infection.
Question 6: Explain the term “eggshell calcified lymph nodes” in the context of silicosis.
Answer 6:
- Silicosis is characterized by chest X-ray findings of “eggshell calcified lymph nodes.”
- This term refers to lymph nodes surrounding the lungs becoming calcified, leading to an eggshell-like appearance on imaging.
Question 7: What types of lung diseases have a predilection for the upper lungs, and which one, including asbestosis, has a predilection for the lower lungs?
Answer 7:
- Silicosis, berylliosis, and coal workers’ pneumoconiosis (CWP) have a predilection for the upper lungs.
- Asbestosis, on the other hand, has a predilection for the lower lungs.
Question 8: Name two lung diseases that asbestos exposure is associated with, besides asbestosis.
Answer 8:
Besides asbestosis, asbestos exposure is associated with
(a) mesothelioma and
(b) bronchogenic carcinoma.
Question 9: What type of rash is seen on the face in sarcoidosis, and how is it described?
Answer 9:
In sarcoidosis, the rash on the face is known as lupus pernio and is characterized by a violaceous appearance.
Question 10: What characteristic feature is seen on chest X-ray in individuals with silicosis, and why is this significant?
Answer 10:
- On chest X-ray, individuals with silicosis exhibit “eggshell” calcified lymph nodes.
- This feature is significant because it helps in identifying and diagnosing silicosis, especially in individuals with a history of sandblasting, mining, or foundry work.
Question 1: List five specific features of Scleroderma and briefly explain each.
Answer 1:
(a) Calcinosis,
(b) Raynaud’s Syndrome (vasospasm of vessels near digits),
(c) Esophageal dysmotility leading to dysphagia and chest pain,
(d) Sclerodactyly causing fibrosis and a “claw-like” appearance of the hands, and
(e) Telangiectasis, which is dilation of superficial skin vessels.
Question 2: What is the main vascular symptom associated with Raynaud’s Syndrome?
Answer 2:
Raynaud’s Syndrome is characterized by vasospasm of the vessels near the digits, leading to color changes in the skin and often causing pain.
Question 3: Explain the term “saddle nose deformity” and its association with which lung disease?
Answer 3:
- Saddle nose deformity is a condition where the bridge of the nose sinks or collapses due to chronic rhinosinusitis.
- It is associated with GPA (Granulomatosis polyangiitis).
Question 4: Describe the common renal symptoms seen in GPA, EPA, and Good Pasture Syndrome.
Answer 4:
All three diseases (GPA, EPA, and Good Pasture Syndrome) present with glomerulonephritis, which includes symptoms like hematuria and proteinuria.
Question 5: What is the characteristic feature associated with Good Pasture Syndrome’s history?
Answer 5:
A history of hemoptysis (coughing up blood) is characteristic of Good Pasture Syndrome.
Question 6: Which lung disease is characterized by fibrosis of the skin of the fingers, leading to a “claw-like” appearance of the hands?
Answer 6:
Sclerodactyly, which is fibrosis of the skin of the fingers, results in a “claw-like” appearance of the hands and is associated with Scleroderma.
Question 7: What is a common gastrointestinal symptom in Scleroderma, and how does it manifest?
Answer 7:
Esophageal dysmotility is a common gastrointestinal symptom in Scleroderma, leading to dysphagia (difficulty swallowing) and chest pain.
Question 8: Describe the term “telangiectasis” and which lung disease it is associated with.
Answer 8:
- refers to the dilation of superficial vessels of the skin.
- This feature is associated with Scleroderma.
Question 9: What are some classical symptoms seen in idiopathic restrictive lung diseases?
Answer 9:
Idiopathic restrictive lung diseases present with classical symptoms such as dyspnea, bibasilar crackles, and dry cough.
Question 10: How do GPA, EPA, and Good Pasture Syndrome present with glomerulonephritis, and how do they differ in other features?
Answer 10:
All three diseases present with glomerulonephritis characterized by hematuria and proteinuria.
They differ in other features,
- chronic rhinosinusitis and saddle nose deformity in GPA,
- asthma or allergic rhinitis history in EPA, and a
- history of hemoptysis in Good Pasture Syndrome.
Question 1: Name and describe the four volumes measured during spirometry in normal lungs.
Answer 1:
The four volumes measured during spirometry in normal lungs are:
(a) Tidal Volume (TV) - the normal inspiration and expiration of air in a single breath,
(b) Inspiratory Reserve Volume (IRV) - the maximum amount of air forcefully inhaled after normal tidal inspiration,
(c) Expiratory Reserve Volume (ERV) - the maximum amount of air forcefully exhaled after normal tidal expiration, and
(d) Residual Volume (RV) - the amount of air remaining in the lungs after ERV.
Question 2: What is the Forced Residual Capacity, and how is it calculated?
Answer 2:
- Forced Residual Capacity is the sum of Expiratory Reserve Volume (ERV) and Residual Volume (RV).
- It is calculated by adding these two volumes together.
Question 3: Define Forced Vital Capacity (FVC) and Total Lung Capacity (TLC) and explain how they are calculated.
Answer 3:
- Forced Vital Capacity (FVC) is the combination of Inspiratory Reserve Volume (IRV),
- Tidal Volume (TV), and Expiratory Reserve Volume (ERV).
- Total Lung Capacity (TLC) is the combination of FVC and Residual Volume (RV).
Question 4: Describe the graph known as the “Flow Volume Loop” and explain how it represents respiratory function.
Answer 4:
- The Flow Volume Loop is a graphical representation of respiratory function done via spirometers.
- It has the X-axis representing volume and the Y-axis representing the flow of air.
- The curve shows both inspiration and expiration.
- During forced deep inhalation (IRV), the curve shows an increase in air volume in the lungs, while during forced deep expiration (ERV), the curve indicates a decrease in air volume back to the original level.
- This graph demonstrates Forced Vital Capacity (FVC), Residual Volume (RV), and Total Lung Capacity (TLC).
Question 5: What does FEV1 represent, and how is it calculated?
Answer 5:
- FEV1 stands for Forced Expiratory Volume at 1 second, which measures the amount of air exhaled after 1 second.
- It is calculated as a ratio of FEV1 to FVC, and this ratio is expressed as a percentage.
Question 6: Calculate the FEV1/FVC ratio given FEV1 = 4L and FVC = 5L.
Answer 6: FEV1/FVC = (4L / 5L) * 100% = 0.8 * 100 = 80%.
Question 7: How is the FEV1/FVC ratio used to assess lung function?
Answer 7:
- The FEV1/FVC ratio is used to assess lung function.
- A lower FEV1/FVC ratio may indicate obstructive lung diseases, whereas a normal or reduced ratio may indicate restrictive lung diseases.
Question 8: How does the FEV1/FVC ratio help distinguish between obstructive and restrictive lung diseases?
Answer 8:
The FEV1/FVC ratio helps distinguish between obstructive and restrictive lung diseases. In obstructive diseases, this ratio is reduced due to difficulty in exhaling air.
In restrictive diseases, the ratio may be normal or reduced, indicating a decrease in lung volumes and capacities.
Question 9: What is the significance of the FEV1/FVC ratio calculation in assessing lung health?
Answer 9:
- The FEV1/FVC ratio calculation is significant in assessing lung health as it helps diagnose and differentiate between obstructive and restrictive lung diseases, providing valuable insights into lung function and potential underlying conditions.
Question 10: Describe the significance of the “saddle nose deformity” and its association with GPA.
Answer 10:
- Saddle nose deformity is a condition where the bridge of the nose sinks or collapses due to chronic rhinosinusitis.
- It is associated with GPA (Granulomatosis polyangiitis), providing a visual clue for the diagnosis of this disease.
Question 1: What is the common issue in all obstructed lung diseases, and how does it affect the lungs?
Answer 1:
- difficulty in getting air out of the lungs.
- This leads to trapped air in the lungs, causing hyperinflation.
- Due to hyperinflation, the lungs cannot take in an additional large volume of air, resulting in a decrease in Inspiratory Reserve Volume (IRV).
- However, Tidal Volume (TV) remains normal, Expiratory Reserve Volume (ERV) increases significantly, and Residual Volume (RV) also increases due to trapped air.
Question 2: Explain why the Forced Vital Capacity (FVC) may be slightly or even normal in obstructed lung diseases.
Answer 2:
- In obstructed lung diseases, although there is a decrease in IRV and an increase in ERV and RV, the FVC may remain slightly or even normal.
- This is because the increased ERV and RV compensate for the decreased IRV, resulting in a maintained or slightly decreased FVC.
Question 3: How does the Flow Volume Loop change in obstructed lung diseases compared to normal lungs?
Answer 3:
- In obstructed lung diseases, the Flow Volume Loop shifts to the left due to increased Residual Volume (RV).
- The graph may also exhibit a coving pattern in the expiratory curve.
Question 4: What does FEV1 stand for, and why is it significantly reduced in obstructed lung diseases?
Answer 4:
FEV1 stands for Forced Expiratory Volume at 1 second.
- In obstructed lung diseases, FEV1 is significantly reduced because of the difficulty in expelling air from the lungs.
Question 5: Calculate the FEV1/FVC ratio given FEV1 = 2L and FVC = 4L.
Answer 5: FEV1/FVC = (2L / 4L) * 100% = 0.5 * 100 = 50%.
Question 6: What is the significance of FEV1/FVC <80% in obstructed lung diseases?
Answer 6:
- An FEV1/FVC ratio less than 80% is a characteristic feature of obstructed lung diseases.
- It indicates that the amount of air forcefully exhaled in the first second (FEV1) is significantly reduced compared to the total volume exhaled (FVC), suggesting airflow obstruction.
Question 7: What are the key characteristics of obstructed lung diseases in terms of the Flow Volume Loop and FEV1/FVC ratio?
Answer 7:
- In obstructed lung diseases, the Flow Volume Loop shifts to the left, and the expiratory curve may exhibit coving.
- The FEV1/FVC ratio is significantly reduced (less than 80%), indicating airflow limitation.
Question 8: How can obstructive lung diseases be distinguished from normal or restrictive lung diseases based on FEV1/FVC ratio?
Answer 8:
- Obstructive lung diseases have a significantly reduced FEV1/FVC ratio (less than 80%),
- whereas normal lung function and restrictive lung diseases typically have FEV1/FVC ratios within or above the normal range.
Question 9: Explain the concept of hyperinflation in obstructed lung diseases and how it affects lung capacity.
Answer 9:
- Hyperinflation occurs in obstructed lung diseases due to trapped air in the lungs.
- This leads to increased Residual Volume (RV) and Total Lung Capacity (TLC), limiting the lung’s ability to fully expand and reducing Inspiratory Reserve Volume (IRV).
Question 10: How does the alteration of volumes and capacities in obstructed lung diseases affect the patient’s ability to breathe?
Answer 10:
- The alteration of volumes and capacities in obstructed lung diseases results in difficulty expelling air from the lungs, leading to trapped air and hyperinflation.
- This reduces the lung’s ability to take in additional air during inspiration and hinders effective breathing.
Question 1: Explain why the lungs are more prone to collapsing in restrictive lung diseases.
Answer 1:
- In restrictive lung diseases, the lungs are very fibrotic and less compliant.
- This makes them more prone to collapsing and limits their ability to hold air.
- Due to reduced compliance, there’s little air inside the lungs, making it hard to bring air in.
- This results in reduced Inspiratory Reserve Volume (IRV), normal Tidal Volume (TV), reduced Expiratory Reserve Volume (ERV) due to low exhalation capacity, and reduced Residual Volume (RV).
- Consequently, Forced Vital Capacity (FVC) significantly decreases, along with Functional Residual Capacity (FRC) and Total Lung Capacity (TLC).
Question 2: Describe the changes in the Flow Volume Loop in restrictive lung diseases compared to normal lungs.
Answer 2:
- In restrictive lung diseases, the Flow Volume Loop will be smaller and shift to the right due to reduced Residual Volume (RV) and significantly decreased FVC.
- There is also a decrease in Total Lung Capacity (TLC).
Question 3: How does the FEV1/FVC ratio change in restrictive lung diseases compared to normal lungs?
Answer 3:
- In restrictive lung diseases, the FEV1/FVC ratio is not as significantly reduced as in obstructed lung diseases.
- While Forced Expiratory Volume at 1 second (FEV1) experiences a mild decrease, Forced Vital Capacity (FVC) significantly drops.
- This results in an FEV1/FVC ratio that is equal to or greater than 80%.
Question 4: Calculate the FEV1/FVC ratio given FEV1 = 3L and FVC = 3.5L.
Answer 4: FEV1/FVC = (3L / 3.5L) * 100% = 0.857 * 100 = 85.7%.
Question 5: How can the FEV1/FVC ratio help differentiate between obstructive and restrictive lung diseases?
Answer 5:
- The FEV1/FVC ratio helps differentiate between obstructive and restrictive lung diseases.
- In obstructive lung diseases, the FEV1/FVC ratio is significantly reduced (less than 80%), while in restrictive lung diseases, it is equal to or greater than 80%.
Question 6: Describe the concept of reduced compliance and its impact on lung function in restrictive lung diseases.
Answer 6:
- Reduced compliance in restrictive lung diseases refers to the lungs’ decreased ability to expand and hold air due to fibrosis.
- This leads to decreased lung volumes, difficulty in inhalation, and limited airflow, ultimately impacting lung function.
Question 7: How do the changes in lung volumes and capacities in restrictive lung diseases affect breathing?
Answer 7:
- The changes in lung volumes and capacities in restrictive lung diseases lead to reduced lung expansion and capacity to hold air.
- This results in difficulties in inhalation and reduced overall lung function, causing patients to experience breathlessness.
Question 8: Explain the relationship between FEV1, FVC, and the FEV1/FVC ratio in obstructed and restrictive lung diseases.
Answer 8:
- In obstructed lung diseases, FEV1 significantly decreases, and the FEV1/FVC ratio is less than 80%.
- In restrictive lung diseases, FVC significantly decreases, and the FEV1/FVC ratio is equal to or greater than 80%.
Question 9: How do the changes in the Flow Volume Loop and FEV1/FVC ratio reflect the altered lung function in restrictive lung diseases?
Answer 9:
- The changes in the Flow Volume Loop (smaller loop shifting to the right) and the FEV1/FVC ratio (≥80%) reflect the reduced lung volumes and capacities in restrictive lung diseases, which result in limited lung expansion, airflow, and overall lung function.
Question 10: What are the key characteristics of restrictive lung diseases in terms of the Flow Volume Loop and FEV1/FVC ratio?
Answer 10:
- In restrictive lung diseases, the Flow Volume Loop shifts to the right due to reduced Residual Volume (RV) and significantly decreased FVC.
- The FEV1/FVC ratio is equal to or greater than 80%, indicating reduced lung volumes but relatively preserved airflow.
Question 1: How can Diffusing Capacity of the Lung for Carbon Monoxide (DLCO) differentiate between intrinsic and extrinsic causes of restrictive lung diseases?
Answer 1:
- DLCO differentiates between intrinsic and extrinsic causes of restrictive lung diseases by measuring the diffusion of lung carbon monoxide.
- In interstitial lung diseases (intrinsic causes), the thickened respiratory membranes lead to a decreased DLCO.
- In extrinsic causes, where there’s no effect on surface area, pressure gradient, or thickness, DLCO remains normal.
Question 2: What are the key factors that DLCO is dependent on and how are they related?
Answer 2:
- DLCO is directly proportional to the Total Surface Area (TSA) and pressure gradient and inversely proportional to the thickness of the respiratory membranes.
- These factors are related in the equation: DLCO ∝ TSA × ΔP / δ.
Question 3: How do Chest X-rays (CXR) help differentiate between different types of interstitial lung diseases?
Answer 3:
- CXRs can differentiate specific types of interstitial lung diseases (ILDs).
- For instance, silicosis, berylliosis, and coal worker’s pneumoconiosis typically affect the upper lobes,
- while asbestosis has a predilection for the lower/basal lobes.
- Sarcoidosis commonly presents with bilateral hilar lymphadenopathy, and
- silicosis is associated with egg-shell calcification of lymph nodes.
Question 4: Describe the CXR findings of reticulonodular opacification.
Answer 4:
- Reticulonodular opacification on CXR appears as reticular opacities moving from the center to the periphery with nodularities.
- This finding is a common occurrence in interstitial lung diseases.
Question 5: What is the significance of the honeycomb sign on CT scans, and in which interstitial lung disease is it commonly observed?
Answer 5:
- The honeycomb sign is a specific CT finding, often seen in idiopathic pulmonary fibrosis (IPF), a type of interstitial lung disease.
- It appears as clustered cystic airspaces with thick walls, resembling a honeycomb pattern, and is typically observed in the periphery of the lungs.
Question 6: Explain the concept of traction bronchiectasis and its association with interstitial lung diseases.
Answer 6:
- Traction bronchiectasis is characterized by dilated bronchioles resulting from intense fibrosis.
- This condition is observed in interstitial lung diseases and is different from the bronchiectasis seen in obstructive lung diseases.
Question 1: What are the confirmatory tests for sarcoidosis, and what abnormalities are typically observed in blood work for sarcoidosis patients?
Answer 1:
The confirmatory tests for sarcoidosis include serum labs, bronchial alveolar lavage (BAL), and biopsy.
In blood work for sarcoidosis patients, there is an elevation of vitamin D, serum calcium (Ca), and angiotensin-converting enzyme (ACE) levels.
Granulomas release 1⍺-hydroxylase, which increases vitamin D levels and subsequently calcium absorption, leading to elevated serum calcium levels. Granulomas also release ACE, contributing to its elevation in blood.
Question 2: How does bronchial alveolar lavage (BAL) help diagnose sarcoidosis, and what does an increased CD4/CD8 ratio in BAL indicate?
Answer 2: Bronchial alveolar lavage (BAL) in sarcoidosis shows an increase in lymphocytes, which leads to an increased CD4/CD8 ratio. This ratio is elevated due to an accumulation of CD4-positive T cells in the lungs, reflecting the immune response characteristic of sarcoidosis.
Question 3: What is the histological finding that confirms sarcoidosis on biopsy, and can this type of granuloma also be seen in another condition?
Answer 3: Biopsy findings that confirm sarcoidosis reveal non-caseating granulomas. It’s important to note that non-caseating granulomas can also be seen in hypersensitivity pneumonitis.
Question 4: What diagnostic clue is suggestive of silicosis, and what histological feature can be seen in lymph node biopsy?
Answer 4: Egg-shell calcifications seen on imaging, along with a strong clinical suspicion, suggest silicosis. Lymph node biopsy in silicosis patients may reveal birefringent silica crystals.
Question 5: What is the significance of Ferruginous bodies in diagnosing asbestos-related diseases?
Answer 5: Ferruginous bodies, observed in sputum samples, are specific markers for asbestos exposure and asbestos-related diseases, including asbestosis, mesothelioma, bronchogenic carcinoma, and pleural plaques.
Question 6: How is berylliosis diagnosed, and what test can confirm the condition?
Answer 6:
Berylliosis is diagnosed by exposure history, clinical presentation, and upper lobe predilection on chest X-ray.
The beryllium lymphocyte proliferation test confirms berylliosis, as exposure of pulmonary fluid (from BAL) to beryllium increases lymphocyte proliferation.
Question 7: What antibodies can be used to confirm the diagnosis of collagen vascular diseases like Scleroderma, EPA, GPA, and Goodpasture Syndrome?
Answer 7: The diagnosis of collagen vascular diseases can be confirmed by testing for specific antibodies. In Scleroderma, the presence of anti-SCL-70 and anti-centromere antibodies can confirm the diagnosis. For EPA, the presence of P-ANCA is indicative, while C-ANCA confirms GPA. In Goodpasture Syndrome, the presence of anti-GBM antibodies is diagnostic.
Question 1: What is the general approach to treating restrictive lung diseases?
Answer 1: The general approach to treating restrictive lung diseases involves addressing the underlying cause or trigger, providing supportive care, reducing inflammation, using antifibrotic medications, and considering lung transplantation if necessary.
Question 2: What are some examples of underlying causes that should be treated in restrictive lung diseases?
Answer 2: Underlying causes that should be treated include environmental exposures (e.g., to barley, hay, pigeon droppings), occupational exposures (e.g., silica, beryllium, coal mines), discontinuing drugs causing lung issues, managing collagen vascular diseases, and addressing idiopathic cases.
Question 3: How can pulmonary rehabilitation benefit patients with restrictive lung diseases?
Answer 3: Pulmonary rehabilitation involves specific exercises to condition and strengthen the lungs, improving their function and the patient’s overall quality of life.
Question 4: When is oxygen supplementation recommended for patients with restrictive lung diseases, and what are the oxygen level criteria?
Answer 4: Oxygen supplementation is recommended when oxygen saturation (SpO2) falls below certain levels. For example, if SpO2 is < 88% and PaO2 is < 55 mmHg, oxygen supplementation is indicated. For patients with cor pulmonale and polycythemia, SpO2 < 90% and PaO2 < 60 mmHg are criteria for oxygen supplementation.
Question 5: How can infections be managed in patients with restrictive lung diseases, and why is infection control important?
Answer 5: Infection control in patients with restrictive lung diseases involves administering pneumococcal and influenza vaccines to prevent infections. Lungs that are already damaged are more susceptible to infections, which can worsen the existing damage. Common infections to prevent include Streptococcus pneumonia, with elderly individuals being at increased risk of Staphylococcus aureus pneumonia.
Question 6: In what situations are steroids and immunosuppressants indicated for the treatment of restrictive lung diseases?
Answer 6: Steroids and immunosuppressants are indicated when patients do not respond adequately to underlying cause treatment and supportive care. They are used to reduce inflammation and prevent further lung injury and fibrosis. These medications are typically used in granulomatous diseases (sarcoidosis, hypersensitivity pneumonitis) and autoimmune diseases (EPA, GPA, scleroderma, Goodpasture).
Question 7: What are antifibrotics, and when are they used in the treatment of restrictive lung diseases?
Answer 7: Antifibrotics, such as pirfenidone and nintedanib, are medications used to target fibroblasts and reduce fibrosis in conditions like idiopathic pulmonary fibrosis (IPF).
Question 8: When is a lung transplant considered as a treatment option for restrictive lung diseases?
Answer 8: A lung transplant is considered a last-line treatment option when a patient with restrictive lung disease is unresponsive to other treatment approaches and experiences severe and irreversible lung damage.
