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RACP Exam Respiratory > Interstitial Lung Disease Update 2023 > Flashcards

Interstitial Lung Disease Update 2023 Flashcards

1. Understanding ILD terminology 2. Basic mgt principles 3. Idiopathic pulmonary fibrosis - focus on this 4. Other important ILDs 5. Short telomeres as a cause of pulmonary fibrosis

1
Q

Features of pulmonary fibrosis radiologically?

A

Reticulation
Traction bronchiectasis
Honeycombing
Architectural distortion
Volume loss

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2
Q

Example of reticulation as feature of pulmonary fibrosis

A
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3
Q

Example of traction bronchiectasis as feature of pulmonary fibrosis

A
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4
Q

Example of honeycombing as feature of pulmonary fibrosis

A

End stage of any form of pulmonary fibrosis

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5
Q

Some radiological features of ILD

A
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6
Q

Why is a good history important for differentiating different types of ILDs?

A

ILD classification is based on different radiological and histological features - and different ILD diseases may have overlap of similar histological/radiological features, so really important to differentiate based on good history

e.g. while IPF is characterised by UIP, UIP is NOT exclusive to this disease

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7
Q

3 different basic management principles of dealing with ILD

A
  • Exposure avoidance +/- immunosuppression
  • Observation +/- immunosuppression
  • Anti-fibrotics i.e. Pirfenidone/Nintedanib
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8
Q

ILDs which tend to be treated with:

Exposure avoidance +/- immunosuppression

A

Pneumoconiosis
Iatrogenic (e.g. Amiodarone, Nitrofurantoin)
Hypersensitivity pneumonitis (birds, mould etc)
Histiocytosis - avoid cigarette smoke!

Smoking-related:
- RB-ILD (Respiratory bronchiolitis interstitial lung disease)
- DIP (Desquamative interstitial pneumonia)

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9
Q

ILDs which tend to be treated with:

Observation +/- immunosuppression

A

Sarcoidosis
Idiopathic LIP (lymphocytic interstitial pneumonia)
COP
NSIP (Non-specific interstitial pneumonia)
CTD-ILD
IPAF

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10
Q

ILDs which tend to be treated with:

Anti-fibrotics i.e. Pirfenidone/Nintedanib

A

IPF and…

Recently however, have data to demonstrate that Nintedanib is effective across a WHOLE RANGE of different ILDs, not just IPF!

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11
Q

Idiopathic pulnonary fibrosis (IPF)
- definition

A

Chronic inexorably progressive fibrosing lung disease of unknown cause

Requires an exclusion of other causes of pulmonary fibrosis (e.g. CTD, drugs, occupational/environmental exposures)

Characterised by UIP histologically (but we use radiological UIP as a strong surrogate for this)

NB: surgical lung biopsy is now rarely required - and does carry a mortality risk!

Importantly, diagnosis MUST be made at an ILD-MDM as this allows access to funding for anti-fibrotic agents

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12
Q

Risk factors for IPF?

A
  • Older age typically 6th/7th decade
    (however beware younger patients with a familial IPF)
  • Male gender 2:1
  • Previous smoking history
  • GORD
  • Family history of ILD
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13
Q

Pre-test probability of IPF based on risk factors

A

The whole point is that ‘idiopathic’ pulmonary fibrosis is just that - IDIOPATHIC

The ‘sqwuaks’ or squeeks on chest auscultation suggests presence of gas trapping which suggests presence of hypersensitifity pneumonitis

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14
Q

Treatment of IPF - previous

A

Prior to 2011 standard trt as through immunosuppression, via combination of
- Azathioprine
- Prednisolone
- N- acetylcysteine

However in 2011 PANTHER study RCT showed that this treatment regimen increased mortality c.f. placebo

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15
Q

Treatment of IPF - current

A

1) AVOIDANCE of immunosuppressants (aside from corticosteroids in acute exacerbations)

2) Anti-fibrotic agent
Pirfenidone
OR
Nintedanib

NB: there has not yet been a head-to-head comparison of the two anti-fibrotic agents but data suggests both associated with slowing of fibrosis as evidenced by measures of reduction of FVC
Also some (not so robust data) that both improve survival

For Nintedanib - some data that reduces incidence of acute exacerbations

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16
Q

Treatment with anti-fibrotic agents makes patients with IPF feel better, T or F?

A

False - these treatments SLOW fibrosis but they cannot reverse it

17
Q

Mechanism of action Pirfenidone

A

Acts through TGF-B and reduces fibroblast proliferation

18
Q

Mechanism of action Nintedanib

A

Inhibits multiple tyrosine kinases (PDGF, VEGF, FGF)

19
Q

Side effects of Pirfenidone

A
  • Photosensitivity rash - strict sun protection!
  • Nausea and other GI upset
20
Q

Side effects Nintedanib

A
  • Possible small increase in bleeding risk - avoid in those on anticoagulants
  • Diarrhoea
  • Weight loss
  • Possible CVS risk
21
Q

What is the role of anti-fibrotic agents OUTSIDE of IPF?

A

Evidence that both have anti-fibrotic effects in other fibrotic lung diseases
- particular evidence for Nintedanib
e.g. in Systemic Sclerosis Associated Interstitial Lung Disease

22
Q

Genetics of IPF?

A

Most common genetic pathway implicated = mutations affecting telomeres -> shortening

Tend to be autosomal dominant pattern of inheritance

Also display genetic anticipation - worsening of phenotype in subsequent generations e.g. first and second generations may have IPF but in 3rd get dyskeratosis congenita in a child (this is caused by critically short telomeres in childhood)

23
Q

Telomeropathy syndrome

A

In high turnover tissues:

  • Premature greying
  • Aplastic anaemia
  • Macrocytosis
  • Thrombocytopenia
  • Opportunistic infections B, T and NK cell immunodeficiency

In slow turnover tissues

  • Pulmonary fibrosis
  • Premature onset emphysema
  • Cryptogenic cirrhosis
  • Osteoporosis
  • Avascular necrosis
  • Epithelial cancers
  • Haematological malignancies
24
Q

Connective Tissue Disease Associated ILD
- which connective tissue diseases does this manifest in?

A

Can manifest in ANY connective tissue disease

However, most common in
-RA
-Systemic sclerosis
-Idiopathic inflammatory myopathies

25
Q

RA-associated ILD has a predilection for which type of histological/radiological pattern?

A

UIP
(Usual Interstitial Pneumonia)

26
Q

Systemic sclerosis- associated ILD has a predilection for which type of histological/radiological pattern?

A

NSIP
(Non-specific Interstitial Pneumonia)
- characterised by diffuse ground glass opacity

27
Q

Idiopathic inflammatory myopathy- associated ILD has a predilection for which type of histological/radiological pattern?

A

OP
(Organising pneumonia)

28
Q

Sjogren syndrome can be associated with which type of ILD?

A

Lymphoid interstitial pneumonia (LIP) - characterised by presence of ground glass opacities and cysts

29
Q

What does this show?

A

Scleroderma-associated ILD with NSIP (non-specific interstitial pneumonia) pattern characterised by diffuse ground glass opacity - and also noted a dilated oesophagus which can be a feature of scleroderma

30
Q

ILD can be a first presentation of a connective tissue disorder (CTD). What is the significance of this?

A

Important in any ILD patient to do a screen for CTD
- ANA
- ENA
- RF
- Anti-CCP
+/- myositis screen
- ANCA - if presentation is with haemoptysis

31
Q

Management of CTD- associated ILD?

A

Immunosuppression

In some cases, can be difficult to distinguish CTD associated ILD from a drug toxicity effect e.g. in many RA patients taking anti-TNF therapy

NB: IMPORTANT to note that although historically blamed, Methotrexate as a cause of ILD has now been largely debunked
So very unlikely that in a patient with RA who has been on stable Methotrexate that the Methotrexate is a cause of their ILD - it is much more likely that they have developed an CTD-associated ILD

32
Q

Hypersensitivity pneumonitis can be non-fibrotic or fibrotic T or F?

A

True

33
Q

What is the histological/radiological pattern most commonly seen in fibrotic hypersensitivity pneumonitis (HP) ?

A

UIP
(Usual interstitial pneumonia)

  • can look very like IPF

Can use HRCT to differentiate if see presence of ground - glass opacity, gas trapping as this points to HP

34
Q

What are some examples of smoking-related ILD?

A
  • RB -ILD (Respiratory bronchiolitis ILD) - characterised by accum of macrophages in bronchioles
  • DIP (Desquamative interstitial pneumonia) - characterised by macrophage infiltration of alveoli
  • Langerhans cell (antigen presenting cells) histiocytosis - characterised by proliferation of abnormal APCs
35
Q

Key to management of smoking-related ILD?

A

Smoking cessation!

36
Q

Sarcoidosis principles of management

A

1) Treat life or organ threatening disease
2) Treat quality of life altering disease - so if life not affected don’t treat!

37
Q

Most common occupational ILDs (Pneumoconiosis) seen in Australia?

A
  • Silicosis (current spike from artificial workbench manufacturing)
  • Asbestosis
  • Coal work’ers pneumoconiosis

Important to take a detailed occupational history in anyone with suspected ILD!

Accurate diagnosis has big impact on compensation/financial wellbeing for patients

38
Q
A

RACP Exam Respiratory (5 decks)

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