Fibrotic Lung Disease

Question 1

Define fibrotic lung disease

Answer 1

Chronic progressive scarring of the lungs leading to a decline in lung function and progressive dyspnoea

Question 2

Aetiology of fibrotic lung disease

Answer 2

Upper lobe fibrosis = TAPE:
T TB
A ABPA
P Pneumoconiosis (silica, coal)
E EAA (hypersensitivity pneumonitis) (mid-zone)

Lower lobe fibrosis = STAIR:
S Sarcoid (mid-zone)
T Toxins (BANS Me)
A Asbestosis
I Idiopathic pulmonary fibrosis(IPF; diagnosis of exclusion)
R Rheumatology (RhA, SLE, Sjogren’s, scleroderma / CREST)

Question 3

Which drugs (Toxins) cause pulmonary fibrosis

Answer 3

B Bleomycin, Busulfan
A Amiodarone
N Nitrofurantoin
S Sulfasalazine
Me Methotrexate

Question 4

Risk factors for pulmonary fibrosis

Answer 4

Age >60
Smoking
Family history
Males
Organic or metal dust
GORD
Diabetes
Viral exposure e.g. HCV, adenovirus, CMV, EBV

Question 5

Symptoms of pulmonary fibrosis

Answer 5

Dyspnoea
- On exertion
- Progressive
- Present for >6 months before presentation
Cough
- Non-productive
- May be severe and non-responsive to anti-tussives
Weight loss, fatigue, malaise, loss of appetite
May precede a viral-type illness

Question 6

Signs of pulmonary fibrosis on examination

Answer 6

End-expiratory basilar fine crackles (almost always)
Clubbing
Advanced - RHF signs (raised JVP, pitting oedema, hepatosplenomegaly, ascites, fatigue)

Question 7

Investigations for pulmonary fibrosis

Answer 7

ABG: normal in early disease → reduced pCO2 with exercise → raised pO2 with late disease
FBC
U&Es
SLE screen (complement, AI screen)
Serum IGE

Spirometry: Restrictive picture
Bronchoalveolar lavage: normal (EAA → cellularity)
CXR: ground glass shadowing → reticulonodular shadowing → honeycombing
CT chest: fibrotic changes “Ground glass shadowing” and “honeycombing”
Echo: ?pulmonary HTN
Bronchoscopy + biopsy: fibrosis

Question 8

Management for pulmonary fibrosis

Answer 8

Conservative:
Physiotherapy
Pulmonary rehabilitation: exercise programme to cope with SOB
Modify risk factors: smoking cessation
Prevention of exacerbations/complications:
- Vaccination (pneumococcal & seasonal flu vaccination)
- Regular exercise (150min/week)
- Avoid being around people with chest infections / colds

Medical:
LTOT
N-acetyl-cysteine → breaks up lung mucous
Pirfenidone OR Nintedanib → reduced rate of fibrosis, anti-oxidant, anti-fibrotic

Surgical
Lung transplant

Question 9

Indications for LTOT in pulmonary fibrosis

Answer 9

pO2 of <7.3 kPa (x2 measurements)
OR
pO2 of 7.3 - 8 kPa and one of:
- Secondary polycythaemia
- Nocturnal hypoxaemia
- Peripheral oedema
- Pulmonary hypertension

Question 10

Management for an acute exacerbation of pulmonary fibrosis

Answer 10

Broad-spectrum ABx and high-dose glucocorticoids

Question 11

Complications of pulmonary fibrosis

Answer 11

Right heart failure
Lung cancer (12%)
Pulmonary embolus
Respiratory failure
Death

Question 12

Prognosis for pulmonary fibrosis

Answer 12

50% died within 3 years of diagnosis
Only 20% survive more than 5 years of diagnosis