Interstitial Lung Disease Flashcards

(57 cards)

1
Q

What is interstitial lung disease (ILD)?

A

It is defined as a group of inflammatory conditions that affect the lung interstitium

This inflammation results in fibrosis formation

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2
Q

What is the lung interstitium?

A

It is the space between an alveolus and its surrounding capillaries

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3
Q

What is the pathophysiological consequence of interstitial lung disease?

A

Due to fibrosis formation, there is increased diffusion distance for oxygen to travel from the alveoli to the surrounding capillaries

Therefore, gas exchange in the lungs is compromised

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4
Q

What are the five classifications of interstitial lung disease?

A

Idiopathic Pulmonary Fibrosis

Secondary Pulmonary Fibrosis

Drug Induced Pulmonary Fibrosis

Hypersensitivity Pneumonitis (HP)

Asbestosis Induced Pulmonary Fibrosis

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5
Q

What is the most common classification of interstitial lung disease?

A

Idiopathic pulmonary fibrosis

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6
Q

What is idiopathic pulmonary fibrosis?

A

It is defined as pulmonary fibrosis with no cause

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7
Q

What are the two risk factors associated with idiopathic pulmonary fibrosis?

A

Male Gender

50 - 70 Years Old

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8
Q

What is secondary pulmonary fibrosis?

A

It is defined as pulmonary fibrosis due to an underlying pathological condition

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9
Q

What are the ten conditions associated with secondary pulmonary fibrosis?

A

Alpha-1 Antitrypsin Deficiency

Rheumatoid Arthritis

Systemic Lupus Erythematous (SLE)

Ankylosing Spondylitis

Systemic Sclerosis

Silicosis

Sarcoidosis

Pneumonia

Tuberculosis

Histiocytosis

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10
Q

What is silicosis?

A

It is a chronic lung disease caused by inhaling large amounts of silica dust - usually related to a mining occupation

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11
Q

What is a CXR feature of silicosis?

A

Egg-shell calcification of hilar nodes

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12
Q

What two pneumonia organisms cause interstitial lung disease?

A

Mycoplasma pneumonia

Pneumocystis pneumonia

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13
Q

What six drugs are associated with interstitial lung disease?

A

Methotrexate

Amiodarone

Bleomycin

Cyclophosphamide

Nitrofurantoin

Radiation Therapy

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14
Q

Which two cancer radiation therapies are associated with interstitial lung disease?

A

Breast Cancer

Lung Cancer

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15
Q

When does interstital lung disease tend to present following radiation therapy course completion?

A

6 - 12 months

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16
Q

What is another term for hypersensitivity pneumonitis (HP)?

A

Extrinsic allergic alveolitis (EAA)

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17
Q

What is hypersensitivity pneumonitis?

A

It is a condition in which hypersensitivity induced lung damage results due to the inhalation of organic particles

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18
Q

What hypersensitivity reaction is associated with hypersensitivity pneumonitis?

A

Type III hypersensitivty

However, type IV is thought to occur in the chronic phase

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19
Q

What are the four allergens associated with hypersensitivity pneumonitis? What are the specific terms used to refer to these conditions?

A

Bird Droppings (Bird-Fanciers Lung)

Hay Mouldy Spores (Farmers Lung)

Mushrooms (Mushroom Workers Lung)

Barley Mould (Malt Workers Lung)

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20
Q

When does the acute phase occur in hypersensitivity pneumonitis, following exposure to an allergen? What are the three acute clinical features?

A

4 - 8 hours

Dyspnoea

Dry Cough

Fever

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21
Q

When does the chronic phase occur in hypersensitivity pneumonitis, following exposure to an allergen? What are the four chronic clinical features?

A

Weeks to Months

Lethargy

Dyspnoea

Productive Cough

Weight Loss

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22
Q

What are two specific investigations used to diagnose hypersensitivity pneumonitis?

A

Blood Tests

Bronchoalveolar Lavage

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23
Q

What two blood test results indicate hypersensitivity pneumonitis?

A

IgG Antibody Positive

No Eosinophilia

24
Q

What bronchoalveolar lavage feature indicates hypersensitvity pneumonitis?

A

Lymphocytosis

25
What are the two management options of hypersensitivty pneumonitis?
To avoid precipitating factors Oral glucocorticoids
26
What is asbestosis induced pulmonary fibrosis?
It is defined as pulmonary fibrosis due to an inhalation of asbestosis
27
Which eight causes of interstital lung disease lead to upper zone fibrosis?
CHARTS Coal Worker's Pneumoconiosis Histiocytosis/Hypersensitivity Pneumonitis Ankylosing Spondylitis Radiation Tuberculosis Silicosis/Sarcoidosis
28
Which four causes of interstital lung disease lead to lower zone fibrosis?
Idiopathic Pulmonary Fibrosis Drug Induced SLE Asbestosis
29
What are the six clinical features associated with interstitial lung disease?
Progressive Dyspnoea Chronic Dry Cough > 3 Months Bibasal Fine -End Inspiratory Crepitations Percussion Dullness Finger Clubbing Raynaud’s Phenomenon
30
What are the four investigations used to diagnose interstitial lung disease?
Spirometry Chest X-Ray (CXR) High-Resolution CT Scan Bronchoscopy
31
What is spirometry?
It involves the conduction of tests that measure the volume and flow of air during exhalation and inhalation
32
What three metrics are obtained from spirometry?
Forced Expiratory Volume 1 (FEV1) Forced Vital Capacity (FVC) FEV1 : FVC
33
What is FEV1?
It is defined as the volume that has been exhaled at the end of the first second of forced expiration
34
What is FVC?
It is is defined as the volume that has been exhaled after a maximal expiration, following a full inspiration
35
What two spirometry results indicates interstitial lung disease?
It is indicated by a restrictive pattern, in which both FVC and FEV1 are reduced, resulting in FEV1 : FVC > 70% Decreased TCLO levels
36
What is the feature of interstitial lung disease on CXR?
Bilateral interstitial shadowing, usually small, irregular peripheral opacities
37
What is the gold standard investigation used to diagnose interstitial lung disease?
High resolution CT scan
38
What are the four features of interstitial lung disease on a high resolution CT scan?
Ground Glass Appearance Honeycombing Traction Bronchiectasis Reticular Opacites
39
What is honeycombing?
It is defined as the cluster of cystic airspaces
40
What is traction bronchiectasis? Why is this a feature of interstitial lung disease?
It is defined as the dilation of airways This is due to the airways being pulled apart by fibrosis in surrounding areas
41
What are reticular opacities? Why is this a feature of interstitial lung disease?
They are defined as pulmonary opacities due to reduced gas exchange This is caused by thickening of the lung interstitium
42
What is bronchoscopy?
It involves the insertion of a small camera into the bronchi
43
How is bronchoscopy used to diagnose interstitial lung disease?
It enables direct visualisation of the parenchymal lung disease It allows a biopsy of this tissue to be obtained for histological examination
44
In hypersensitivity pneumonitis, what two bronchoscopy histology results are shown?
Increased lymphocytes Mast cells
45
In what five ways do we conservatively manage interstitial lung disease?
Underlying Pathology Management Smoking Cessation Pulmonary Rehabilitation Annual Influenza Vaccination One-Off Pneumococcal Vaccination
46
What classification of interstitial disease can be pharmacologically managed?
Idiopathic pulmonary fibrosis
47
What are the two pharmacological options for idiopathic pulmonary fibrosis?
Pyridones Monoclonal Antibodies
48
Name a pyridone used to manage idiopathic pulmonary fibrosis
Pirfenidone
49
What is the mechanism of action of pirfenidone?
It is an antifibrotic and anti-inflammatory that is administered to slow down the progression of disease
50
Name a monoclonal antibody used to manage idiopathic pulmonary fibrosis
Nintedanib
51
What is the mechanism of action of nintedanib?
It inhibits the action of tyrosine kinase which are involved in the formation of fibrosis
52
In what other circumstance can we pharmacologically manage interstitial lung disease? What drugs can be administered?
In cases where the underlying cause is related to connective tissue diseases or sarcoidosis Steroids and steroid sparing agents
53
What steroid is used to manage interstitial lung disease related to connective tissue diseases or sarcoidosis?
Prednisolone
54
What two steroid sparing agents are used to manage interstitial lung disease related to connective tissue diseases or sarcoidosis?
Azathioprine Mycophenolate
55
What criteria is used to determine whether interstitial lung disease patients should be administered LTOT?
Resting PaO2 < 7.3kPa OR Resting PaO2 < 8kPa With Peripheral Oedema, Polycythaemia, Pulmonary Hypertension
56
What surgical management option can be used in interstitial lung disease?
Lung transplant
57
When is lung transplant considered to manage interstitial lung disease?
It is only considered in individuals with severely impacted quality of life, despite optimum pharmacological management