Interstitial lung disease

Question 1

What are some clinical signs of ILD

Answer 1

SOB
Clubbing (IPF)
Central cyanosis
Tracheal deviation towards effected side
Flat non expansile chest
Scars
Increased tactile vocal fremitus + resonance
Dull to percussion
Pulmonary HTN

Question 2

What may you hear on auscultation on ILD

Answer 2

Fine late inspiratory crackles
Squarks (hypersensitivity pneumonitis)
Crackles may disappear on leaning forwards
Unaffected by cough (unlike pulmonary oedema/ infection)
Fine in nature NOT coarse

Question 3

What signs may be present in the hands/ arms

Answer 3

Rhematic changes
rheumatic nodules
Sclerodactyly (diffuse cutaneous Scl-70)
Clubbing

Question 4

Causes of apical fibrosis

Answer 4

CHARTS

Coal worker pneumoconiosis
Histoplasmosis/ hypersensitivity
Ank Spond/ ABPA
Radiation
TB
Silicosis/ Sarcoidosis

Question 5

Causes of basal fibrosis

Answer 5

Asbestosis
Connective tissues (Sceloderma/ SLE/ Sjogrens/ dermatomyositis/Rheumatoid)
Drugs (Bleomycin/ MTX/ amiodarone/ nitrofurantoin)
Pulmonary fibrosis (idiopathic)

Question 6

Types of idiopathic interstitial pneumonias

Answer 6

Usual interstitial pneumonia - defines IPF, Also seen in asbestosis, chronic hypersensitivity pneumonitis, collagen vascular disease and chronic drug toxicity. 10% steroid responsive

Non-specific interstitial pneumonia - typically steroid responsive (60%). better prognosis than UIP.

Acute interstitial pneumonia (Hammond rich syndrome) - acute rapid progressive fibrosis, poor prognosis

Desquamative interstitial pneumonia
Cryptogenic organising pneumonia

Question 7

Differentials of ILD?

Answer 7

pulmonary oedema
bilateral bronchiectasis
bilateral pneumonias

Question 8

How is ILD investigated

Question 9

How can rheumatoid arthritis effect the lung?

Answer 9

Pulmonary nodules (seropositive patients) - more likely have nodules elsewhere too)
Caplan’s syndrome
Pulmonary fibrosis ( UIP>NSIP (more ground glass) ). poor prognosis
Obliterative bronchiolitis
Pleural effusion

Question 10

Management of interstitial lung disase

Answer 10

MDT approach
physio/ OT
Pulmonary rehab
Definitive management depends on cause. for example, drug induced = remove causative drug. RA = management of the RA. steroids is connective tissues NSIP type

Question 11

What is the medical management of IPF

Answer 11

Pirfenidone - antifibrotic agent
Nintedanib (tyrosine kinase inhibitor)
LT Oxygen therapy (paO2 <7.3KPa or <8Kpa with signs of cor pulmonale)

Question 12

What is the surgical management of ILD?

Answer 12

Patient’s could be considered for lung transplant for example stage 4 sarcoid with fibrosis

Question 13

How should ILD be investigated

Answer 13

Should be investigated as per of an MDT approach given diagnosis made in conjunction between expert.

• Bloods (Routine + specifically thinking of connective tissue so ENA/ ANA/ immunoglobulins/ serum ACE/ RF/ DsDNA/ C3+4)
• ABG
• Lung function tests
• CXR (reticulonodular shadowing)
• HRCT (honeycombing/ ground glass changes)
• bronchioalveolar lavage
• Echocardiogram (pulmonary HTN)
• 6 minute walk test
• Biospy - if imaging inconclusive

Question 14

What are the features of ILD on LFTs

Answer 14

Restrictive pattern
FEV1:FVS >0.7 (preserved ratio)
Numbers lower than predicted
reduced TLCO (important prognostic factor)

Question 15

When would lymphocytes be found on BAL

Answer 15

Sarcoidosis
Hypersensitivity pneumonitis
Organising pneumonia
lymphocytic interstitial pneumonia

Question 16

CXR features and stages of sarcoidosis

Answer 16

1. Liar (hilar lymphadenopathy)
2. Liar pants (hilar lymphadenopathy + Pulmonary infiltrates)
3. On (Pulmonary infiltrates)
4. Fire (fibrosis)

Question 17

Why do patients with COPD do poorly with single lung transplant?

Answer 17

Native lung hyperinflates. seeding of infection from native lung