Interstitial Lung Disease Flashcards

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the lung interstitium?

A

It is the space between an alveolus and its surrounding capillaries

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the most common classification of interstitial lung disease?

A

Idiopathic pulmonary fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is idiopathic pulmonary fibrosis?

A

It is defined as pulmonary fibrosis with no cause

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

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

A

Male Gender

50 - 70 Years Old

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is secondary pulmonary fibrosis?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is a CXR feature of silicosis?

A

Egg-shell calcification of hilar nodes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What two pneumonia organisms cause interstitial lung disease?

A

Mycoplasma pneumonia

Pneumocystis pneumonia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What six drugs are associated with interstitial lung disease?

A

Methotrexate

Amiodarone

Bleomycin

Cyclophosphamide

Nitrofurantoin

Radiation Therapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

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

A

Breast Cancer

Lung Cancer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

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

A

6 - 12 months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is another term for hypersensitivity pneumonitis (HP)?

A

Extrinsic allergic alveolitis (EAA)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What are two specific investigations used to diagnose hypersensitivity pneumonitis?

A

Blood Tests

Bronchoalveolar Lavage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
Q

What are the two management options of hypersensitivty pneumonitis?

A

To avoid precipitating factors

Oral glucocorticoids

26
Q

What is asbestosis induced pulmonary fibrosis?

A

It is defined as pulmonary fibrosis due to an inhalation of asbestosis

27
Q

Which eight causes of interstital lung disease lead to upper zone fibrosis?

A

CHARTS

Coal Worker’s Pneumoconiosis

Histiocytosis/Hypersensitivity Pneumonitis

Ankylosing Spondylitis

Radiation

Tuberculosis

Silicosis/Sarcoidosis

28
Q

Which four causes of interstital lung disease lead to lower zone fibrosis?

A

Idiopathic Pulmonary Fibrosis

Drug Induced

SLE

Asbestosis

29
Q

What are the six clinical features associated with interstitial lung disease?

A

Progressive Dyspnoea

Chronic Dry Cough > 3 Months

Bibasal Fine -End Inspiratory Crepitations

Percussion Dullness

Finger Clubbing

Raynaud’s Phenomenon

30
Q

What are the four investigations used to diagnose interstitial lung disease?

A

Spirometry

Chest X-Ray (CXR)

High-Resolution CT Scan

Bronchoscopy

31
Q

What is spirometry?

A

It involves the conduction of tests that measure the volume and flow of air during exhalation and inhalation

32
Q

What three metrics are obtained from spirometry?

A

Forced Expiratory Volume 1 (FEV1)

Forced Vital Capacity (FVC)

FEV1 : FVC

33
Q

What is FEV1?

A

It is defined as the volume that has been exhaled at the end of the first second of forced expiration

34
Q

What is FVC?

A

It is is defined as the volume that has been exhaled after a maximal expiration, following a full inspiration

35
Q

What two spirometry results indicates interstitial lung disease?

A

It is indicated by a restrictive pattern, in which both FVC and FEV1 are reduced, resulting in FEV1 : FVC > 70%

Decreased TCLO levels

36
Q

What is the feature of interstitial lung disease on CXR?

A

Bilateral interstitial shadowing, usually small, irregular peripheral opacities

37
Q

What is the gold standard investigation used to diagnose interstitial lung disease?

A

High resolution CT scan

38
Q

What are the four features of interstitial lung disease on a high resolution CT scan?

A

Ground Glass Appearance

Honeycombing

Traction Bronchiectasis

Reticular Opacites

39
Q

What is honeycombing?

A

It is defined as the cluster of cystic airspaces

40
Q

What is traction bronchiectasis? Why is this a feature of interstitial lung disease?

A

It is defined as the dilation of airways

This is due to the airways being pulled apart by fibrosis in surrounding areas

41
Q

What are reticular opacities? Why is this a feature of interstitial lung disease?

A

They are defined as pulmonary opacities due to reduced gas exchange

This is caused by thickening of the lung interstitium

42
Q

What is bronchoscopy?

A

It involves the insertion of a small camera into the bronchi

43
Q

How is bronchoscopy used to diagnose interstitial lung disease?

A

It enables direct visualisation of the parenchymal lung disease

It allows a biopsy of this tissue to be obtained for histological examination

44
Q

In hypersensitivity pneumonitis, what two bronchoscopy histology results are shown?

A

Increased lymphocytes

Mast cells

45
Q

In what five ways do we conservatively manage interstitial lung disease?

A

Underlying Pathology Management

Smoking Cessation

Pulmonary Rehabilitation

Annual Influenza Vaccination

One-Off Pneumococcal Vaccination

46
Q

What classification of interstitial disease can be pharmacologically managed?

A

Idiopathic pulmonary fibrosis

47
Q

What are the two pharmacological options for idiopathic pulmonary fibrosis?

A

Pyridones

Monoclonal Antibodies

48
Q

Name a pyridone used to manage idiopathic pulmonary fibrosis

A

Pirfenidone

49
Q

What is the mechanism of action of pirfenidone?

A

It is an antifibrotic and anti-inflammatory that is administered to slow down the progression of disease

50
Q

Name a monoclonal antibody used to manage idiopathic pulmonary fibrosis

A

Nintedanib

51
Q

What is the mechanism of action of nintedanib?

A

It inhibits the action of tyrosine kinase which are involved in the formation of fibrosis

52
Q

In what other circumstance can we pharmacologically manage interstitial lung disease? What drugs can be administered?

A

In cases where the underlying cause is related to connective tissue diseases or sarcoidosis

Steroids and steroid sparing agents

53
Q

What steroid is used to manage interstitial lung disease related to connective tissue diseases or sarcoidosis?

A

Prednisolone

54
Q

What two steroid sparing agents are used to manage interstitial lung disease related to connective tissue diseases or sarcoidosis?

A

Azathioprine

Mycophenolate

55
Q

What criteria is used to determine whether interstitial lung disease patients should be administered LTOT?

A

Resting PaO2 < 7.3kPa

OR

Resting PaO2 < 8kPa With Peripheral Oedema, Polycythaemia, Pulmonary Hypertension

56
Q

What surgical management option can be used in interstitial lung disease?

A

Lung transplant

57
Q

When is lung transplant considered to manage interstitial lung disease?

A

It is only considered in individuals with severely impacted quality of life, despite optimum pharmacological management