Restrictive Lung Diseases (L19) Flashcards
List the features of Restrictive Lung diseases.
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- Increased Lung Density
- Lung is Stiff
- Reduced Compliance
- Breathlessness (Dyspnoea)
- Greater Effort to Inflate Lungs
- Abnormality of Alveolar Walls which renders them rigid
An increase in interstitial fluid or fibrosis produces what?
A Stiff lung.
What is ARDS?
What is it characterised by?
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- ARDS is a syndrome of acute and persistent inflammatory disease of the lungs and is characterised by:
- Bilateral inflammatory infiltrates
- Ratio of partial pressure of arterial oxygen to the fractional concentration of inspired air (Pa02/FiO2) of less than 200 mmHg.
List the risk factors of ARDS.
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- Sepsis (the most common cause)
- Massive trauma with shock and multiple transfusions
- Hypovolaemic shock (the volume of circulatory sytstem is depleted thus there is limited circulation to the tissues)
- Pneumonia
- Smoke Inhalation
- Drugs – paraquat, heroin, asprin
List the features of ARDS.
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- Diffuse alveolar damage
- Hyaline membranes line the respiratory
bronchioles - Severe injury to alveolar capillary walls
- Tachypnea (abnormally fast breathing) and Dyspnoea.
- Pulmonary Oedema
- Arterial Hypoxaemia
- Alveolar flooding and Atelectasis (due to loss of surfactant)
What is the ARDS mortality rate?
40%.
When a person has Interstitial Lung disease (ILD) the lung is affected in what three ways?
- Lung tissue is damaged
- Walls of the acini become inflamed
- Fibrosis begins in the interstitium and the lungs become stiff
_____ _____ _____ is a common term for more than 130 chronic lung diseases.
Interstitial Lung Disease.
Name the disease grouping.
- These develop over months/years and leads to a slowly decreasing respiratory efficiency.
- With chronic interstitial fibrosis leads to a ‘honeycomb’ lung.
- There is an infiltration of macrophages and microcyst formation.
- Clinically patients exhibit dyspnoea, cough and in advanced cases hypoxemia and cyanosis.
Chronic Restrictive Lung Diseases.
___1___ lung disease has traditionally been thought to occur as a result of an initial injury to the lung that causes the recruitment of ___2___ cells, release of cytokines and eventually, from increased fibroblast activity parenchymal remodelling and ___3___.
- Interstitial
- Inflammatory
- Fibrosis
In terms of Interstitial Lung Diseases;
Inflammation of the bronchioles=?
Bronchiolitis.
In terms of Interstitial Lung Diseases;
Inflammation of the alveoli=?
Alveolitis.
In terms of Interstitial Lung Diseases;
Inflammation of the capillaries=?
Vasculitis.
Idiopathic pulmonary fibrosis (IPF) affects about __/100 000 people.
Otherwise known as?
15.
Cryptogenic fibrosing alveolitis.
List the 4 types of Idiopathic pulmonary fibrosis (IPF).
- Usual Interstitial Pneumonitis (UIP)
- Desquamative Interstitial Pneumonitis (DIP)
- Acute Interstitial Pneumonitis (AIP)
- Non-Specific Interstitial Pneumonitis
Describe the most outstanding feature of Usual Interstitial Pneumonitis (UIP).
Temporal heterogeneity meaning a biopsy obtained on a given day may show two or more different developmental features plus a non pathological feature (normal lung interspersed with diseased areas).
List the symptoms of Usual Interstitial Pneumonitis (UIP).
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- Persistent cough
- Dyspnoea
- Fatigue
- Fever
- Joint symptoms are common and finger clubbing occurs in 50% of cases.
Describe the most outstanding feature of Desquamative Interstitial Pneumonitis (DIP).
The presence of large numbers of macrophages in the alveolar spaces, some with a brown cytoplasmic pigmentation.
List the symptoms of Desquamative Interstitial Pneumonitis (DIP).
(3)
- Slow progressive dyspnea
- Non-productive cough
- Clubbing
What is the other name for Acute Interstitial Pneumonitis (AIP)?
Hamman-Rich syndrome.
Describe the most outstanding feature of Acute Interstitial Pneumonitis (AIP).
Rapidly progressive interstitial pulmonary fibrosis.
List the symptoms of Acute Interstitial Pneumonitis (AIP).
- Rapidly progressive dyspnoea
- Hypoxemia
- Respiratory failure
Histologically, what is the hallmark of Acute Interstitial Pneumonitis (AIP)?
Describe.
- Diffuse alveolar damage (DAD)
- DAD manifests initially as injury to the alveolar lining and endothelial cells, pulmonary oedema and hyaline membrane formation. On X-ray there is patchy airspace opacifications
Describe the most outstanding feature of Non-Specific Interstitial Pneumonitis.
Diffuse interstitial thickening with inflammatory cells and fairly good preservation of the alveolar architecture.
Describe Bronchiolitis Obliterans.
Characterised by formation of fibrous tissue within the lumina of small bronchioles and alveoli. Very sensitive to corticosteroid therapy.
Describe Sarcoidosis.
A relatively common granulatomatas disease. In many cases the disease is asymptomatic although lung destruction and fibrosis may occur.
