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.
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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.”
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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.