Interstitial Lung Disease Flashcards

1
Q

What is interstitial lung disease?

A

Group of conditions that cause inflammation +/or fibrosis of the lung parenchyma

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2
Q

What is fibrosis?

A

Replacement of elastic, functional lung tissue with non-functional scar tissue

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3
Q

Give 5 examples of interstitial lung disease

A

Idiopathic pulmonary fibrosis
Secondary pulmonary fibrosis
Hypersensitivity pneumonitis
Cryptogenic organising pneumonia
Pneumoconiosis (inc. Asbestosis)

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4
Q

Give 3 symptoms of interstitial lung disease

A

SOBOE
Dry cough
Fatigue

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5
Q

Describe idiopathic pulmonary fibrosis. What is the life expectancy?

A

Progressive pulmonary fibrosis with no apparent cause
Life expectancy: 2-5y

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6
Q

Give 2 signs of IPF

A

Bibasal fine end-inspiratory crepitations
Clubbing

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7
Q

Describe management of IPF

A

C: Pulmonary rehabilitation + stop smoking
M: Pirfenidone (reduces fibrosis + inflammation) + LTOT
S: lung transplant

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8
Q

Name 4 drugs that can cause secondary pulmonary fibrosis

A

Amiodarone (+ grey/ blue skin)
Cyclophosphamide
Methotrexate
Nitrofurantoin

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9
Q

Name 5 conditions that can cause secondary pulmonary fibrosis

A

Alpha-1 antitrypsin deficiency
RhA
SLE
Systemic sclerosis
Sarcoidosis

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10
Q

What is hypersensitivity pneumonitis also known as?

A

Extrinsic allergic alveolitis

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11
Q

What is hypersensitivity pneumonitis?

A

Inflammation of alveoli + distal bronchioles caused by an immune response to inhaled allergens

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12
Q

What immune response occurs in hypersensitivity pneumonitis?

A

Type III + iV hypersensitivity

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13
Q

Give 4 examples of hypersensitivity pneumonitis

A

Bird fanciers’ lung
Farmers lung
Malt workers’ lung
Mushroom workers’ lung

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14
Q

List 5 symptoms in acute hypersensitivity pneumonitis

A

Cough
Dyspnoea
Fever
Malaise
Chest tightness

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15
Q

List 4 ways in which chronic hypersensitivity pneumonitis manifests

A

Insidious cough/ dyspnoea
Weight loss
Clubbing
Widespread fibrotic changes mimicking IPF

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16
Q

What will be heard on auscultation in hypersensitivity pneumonitis?

A

Bilateral mid-zone inspiratory crepitations

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17
Q

What investigations are performed in suspected hypersensitivity pneumonitis ?

A

FBC: r/o infection
Serum precipitant antibodies
CXR
HRCT
Lung function tests
Bronchoalevolar lavage

18
Q

What may be seen on CXR in hypersensitivity pneumonitis?

A

Ground glass changes
Airspace consolidation
Interstitial opacities

19
Q

What 5 features may be seen on HRCT in hypersensitivity pneumonitis?

A

Patchy, diffuse, symmetrical ground glass opacities
Small (<5mm) centrilobular opacities
Patchy air trapping on expiratory imaging
Airspace consolidation
Changes are typically bilateral in the mid-zone

20
Q

Describe lung function tests in hypersensitivity pneumonitis

A

Acute: Restrictive pattern
Chronic: mixed pattern

TLCO reduced

21
Q

What may be found on bronchoalveolar lavage in hypersensitivity pneumonitis?

A

Antibodies to causative antigen
Lymphocytosis

22
Q

Describe first line management of hypersensitivity pneumonitis

A

Identification + removal of causative agent

23
Q

How can corticosteroids be used in hypersensitivity pneumonitis?

A

Differentiate dx: HP tends to be steroid responsive, IPF does not
Symptomatic relief

24
Q

Which patients with hypersensitivity pneumonitis may benefit from long term steroids?

A

Chronic HP may benefit from long term low-dose Prednisolone

25
Q

What are the complications of hypersensitivity pneumonitis?

A

Fibrotic + emphysematous changes following inflammation may lead to fibrosis
May need LTOT
Reduced ability to perform ADLs

26
Q

What is cryptogenic organising pneumonia (COP)?

A

diffuse ILD that affects distal bronchioles, respiratory bronchioles, alveolar ducts + alveolar walls

27
Q

List 4 symptoms of COP

A

Cough
SOB
Fever
Malaise

28
Q

What may be seen on bloods in COP?

A

Leukocytosis
High ESR + CRP

29
Q

What is seen on imaging in COP?

A

CXR: Bilateral patchy infiltrates, diffuse consolidative or ground glass opacities
HRCT: triangle sign, air-bronchograms

30
Q

What is the distinctive triangle sign commonly seen in COP?

A

Triangular ground glass opacity with the base on the pleura + apex towards the mediastinum

31
Q

Describe lung function tests in COP

A

Classically restrictive pattern
(sometimes obstructive or normal)
TLCO reduced

32
Q

Describe management of COP

A

Mild: watch + wait
Severe: high dose (40-60mg) Prednisolone PO

33
Q

What is asbestosis?

A

Lung fibrosis related to asbestos exposure

34
Q

What are pneumoconioses?

A

Group of lung diseases caused by inhalation of harmful dusts
(aka occupational ILDs)

35
Q

Give 4 examples of pneumoconioses

A

Asbestosis
Silicosis
Coal workers pneumoconiosis (black lung disease)
Chronic beryllium disease (Berylliosis)

36
Q

Give 2 features of silicosis on CXR

A

Progressive upper zone fibrosis
“Egg shell” calcification of the hilar LNs

37
Q

Describe CXR in coal workers pneumoconiosis

A

Progressive upper zone fibrosis

38
Q

Describe CXR in berylliosis

A

Progressive upper zone linear interstitial fibrosis

39
Q

Describe lung function tests in pneumoconioses

A

May show restrictive, obstructive or normal pattern

40
Q

Describe conservative management of pneumoconioses

A

Smoking cessation
Removal of occupational exposure
Advice regarding compensation
+/- pulmonary rehabilitation

41
Q

What medication is used in berylliosis?

A

Prednisolone 40-70mg PO