ILD

Question 1

What is ILD

Answer 1

Disease affecting tissue and alveoli of the lung. Characterised by diffuse inflammation and/or progressive interstitial fibrosis

Question 2

What are the signs and symptoms of ILD

Answer 2

SOB on exertion 
non-productive paroxysmal cough (paroxysmal = intense for short periods of time)
restrictive pattern on spirometry
Decreased DLCO  (decreased lung capacity for CO)

Question 3

What are the signs of fibrosis

Answer 3

Clubbing
Dry cough
Fine inspiratory crackles
Cyanosis

Question 4

Which drugs can cause pulmonary fibrosis?

Answer 4

Methotrexate
Nitrofuratoin
Bleomycin
Amiodarone

Question 5

What investigations are done for patients with suspected ILD

Answer 5

Bedside obs 
- sats = oxygen sats may be low
- RR = resp rate will be high
Lung function tests - shows restrictive deficit. LOw FVC but FEV1 may be normal or increased 
Bloods - raised CRP 
ABG shows decreased pO2
CXR - shows ground glass appearance 
CT - honeycomb appearance 
Biopsy and histology to confirm

Question 6

What does a CXR show in ILD

Answer 6

Reticular shadowing at the lung peripheries more prominent towards lung bases
Diffuse opacification throughout affected areas of the lung - ground glass appearance

Question 7

What is the management for ILD

Answer 7

Refer to specialist ILD clinic

• Oxygen if needed
• Pulmonary rehab
• Smoking cessation
• Palliative care
• Prenisolone, azathioprine and nebulsied N-acetylcysteine to help loosen up mucus
• removal of risk factors

Question 8

Following diagnosis of ILD which other investigations should be done?

Answer 8

Tests for

• SLE - ANA and Anti-dsDNA
• Rheumatoid factor
• ANCA
• Anti GBM for goodpastures disease

Question 9

What is the pathophysiology behind ILD

Answer 9

It is thought that acute injury of the lung parenchyma occurs –> chronic inflammation –> fibroblast activation and proliferation –> increase in fibrous tissue and tissue destruction
Making it more difficult for oxygen to diffuse across the alveolar membrane into the capillaries

Question 10

What are the risk factors for developing ILD

Answer 10

Smoking
Keeping birds
Occupational exposure - asbestos, silicone
drug induced - methotrexate, nitrofuratoin, amiodarone, bleomycin
previous infection

Question 11

What other conditions can cause a restrictive pattern on spirometry

Answer 11

Neuromuscular - motor neurone disease
Chest deformities - kyphosis, pectus carinatum, pectus excavatum
Obesity
ARDS

Question 12

How many Idiopathic pulmonary fibrosis differ from other causes of ILD?

Answer 12

It may present with:

Weight loss
Malaise
Arthralgia

Question 13

Which drugs should NOT be used in idiopathic pulmonary fibrosis

Answer 13

High dose steroids

Question 14

What is the mnagement for idiopathic pulmonary fibrosis

Answer 14

Oxygen
Pulmonary rehab 
opiates 
palliative care input 
All patients should be considered for clinical trials or lung transplant

Question 15

What are the 3 categories ILD can be catergorised into?

Answer 15

Those with known cause

• Occupational/environmental
• Drugs e.g. nitrafuratoin, methotrexate, bleomycin, amiodarone, sulfalazine
• Hypersensitivity reactions e.g. extrinsic allergic alveolitis
• infection e.g. TB, fungi, viral

Those with systemic disease

• RA
• SLE
• Sarcoidosis
• Systemic sclerosis
• UC, Renal tubular acidosis, autoimmune thyroid disease

Idiopathic

• Idiopathic pulmonary fibrosis
• Cryptogenic organising pneumonia
• Lymphocytic interstitial pneumonia

Question 16

Which idiopathic pulmonary fibrosis has the worse prognosis

Answer 16

Usual interstital Pneumonia

20-30% survival rate

Question 17

What are the hallmarks of UIP

Answer 17

subpleural distribution of ground glass and reticulonodular changes on high resolution CT
and
temporal heterogeneity of changes on biopsy