ILD, EAA, IPF

Question 1

What is interstitial lung disease

Answer 1

Generic term used to describe a number of conditions that primarily affect the lung parenchyma in a diffuse manner

Chronic inflammation and/or progressive interstitial fibrosis

Share a number of clinical and pathological features

Question 2

What are the clinical features of ILD

Answer 2

Dyspnoea on exertion
Non-productive paroxysmal dry cough

Abnormal breath sounds

Chest pain - increased risk of pneumothorax due to fibrosis

Clubbing

Question 3

Pathological features of ILD

Answer 3

Chronic inflammation
Fibrosis and remodelling of interstitium
Hyperplasia of type II epithelial cells or type II pneumocytes

Question 4

3 categories of ILD

Answer 4

Known Cause
Associated with systemic disease
Idiopathic

Question 5

Idiopathic causes of ILD

Answer 5

Idiopathic pulmonary fibrosis
Cryptogenic organising pneumonia
Lymphocytic interstitial pneumonia

Question 6

ILD associated with systemic disorders

Answer 6

Sarcoidosis 
RA
SLE 
UC 
Connective tissue disease 
Autoimmune thyroid disease

Question 7

Known causes of ILD

Answer 7

Occupational/environmental 
Drugs 
Hypersensitivity reactions (EAA)
Infections eg. TB, fungal or viral 
GORD

Question 8

ILD CXR

Answer 8

Patchy shadowing

Question 9

HR CT ILD

Answer 9

Fibrosis

Honeycombing - IPF

Question 10

What is Extrinsic Allergic Alveolitis

Answer 10

Inhalation of allergens (fungal spores or avian proteins) causes a hypersensitivity reaction in sensitised individuals

Question 11

What happens in acute and chronic phase of EAA?

Answer 11

Acute - alveoli infiltrated with acute inflammatory cells

Chronic - granuloma formation and obliterative bronchiolitis

Question 12

Causes of EAA - the sensitizing thing

Answer 12

Bird fanciers and pigeon fanciers lung - proteins in bird droppings

Farmers and mushroom workers lung

Malt workers lung

Sugar workers lung

Question 13

Clinical features 4-6 hour post exposure to allergen in EAA

Answer 13

Fever, rigours, myalgia, dry cough, dyspnoea, crackles but no wheeze
Inspiratory crepitations

Question 14

Chronic features of EAA

Answer 14

Increasing dyspnoea and decreasing exercise tolerance 
Weight loss
type 1 respiratory failure 
cor pulmonale 
Inspiratory crepitations

Question 15

CXR in acute and chronic EAA

Answer 15

ACUTE
Upper-zone mottling/consolidation

CHRONIC
upper lobe fibrosis
Honeycomb lung

CT
Ground glass appearance

Bronchoalveolar lavage - CD8 T-lymphocytes and mast cells

Question 16

Lung function tests in EAA

Answer 16

Restrictive - therefore FVC decrease more than FEV1

Decreased total lung capacity

Question 17

Management of EAA acutely

Answer 17

Remove allergen and give O2 - acutely

Then oral prednisolone

Question 18

Long term managment of EAA

Answer 18

Avoid exposure to allergens
Wear a facemask or +ve pressure helmet if unavoidable

Long-term steroids may improve CXR and physiology but not always - 1month trial

Compensation?!?

Question 19

Causes of upper zone fibrotic shadowing on CXR

Answer 19

TB
EAA
Ankylosing spondylitis 
Radiotherapy 
Sarcoid

Question 20

Causes of middle zone fibrotic shadowing on CXR

Answer 20

Progressive massive fibrosis

Question 21

Causes of lower zone shadowing on CXR

Answer 21

IPF

Asbestosis

Question 22

What are the 5 ways that the aspergillus fungi affect the lung

Answer 22

1) Asthma - type 1 hypersensitivity reaction to fungal spores
2) Allergic bronchopulmonary aspergillosis
3) Aspergilloma
4) Invasive aspergillosis
5) Extrinsic Allergic alveolitis - ‘malt workers lung’ sensitivity to this fungi

Question 23

What is allergic bronchopulmonary aspergillosis?

Answer 23

Hypersensitivity to aspergillus fungus
Causes bronchoconstriction but then inflammation and permanent damage occurs causing bronchiectasis
Wheeze, cough, sputum, dyspnoea and ‘recurrent pneumonia’

Question 24

Treatment of allergic bronchopulmonary aspergillosis ABPA

Answer 24

Prednisolone for acute attacks 30-40mg
Maintenance dose of 5-10mg
Sometimes itraconazole

Question 25

What is aspergilloma?

Answer 25

Fungal ball within a pre-existing cavity (TB or sarcoid) Usually asymptomatic Can cause cough with haemoptysis Also lethargy and weight loss Treatment is removal if solitary symptomatic or lots of haemoptysis

Question 26

What is invasive aspergillosis?

Answer 26

Invasion of aspergillus into lung tissue and fungal dissemination to other parts of the body in immunocompromised patients dyspnoea, rapid deterioration and septic picture Treat with voriconazole or other -zole's

Question 27

What is Idiopathic Pulmonary Fibrosis?

Answer 27

Type of idiopathic interstitial lung disease Inflammatory cell infiltrate and lung fibrosis - unknown cause Also called idiopathic fibrosing alveolitis and previously called cryptogenic fibrosing alveolitis

Question 28

Signs of IPF

Answer 28

Dry cough, no wheeze, gradual onset of exertional dyspnoea, clubbing, cyanosis, fine end-inspiratory creps Malaise + fatigue, weight loss and arthralgia

Question 29

Complications associated with IPF

Answer 29

Resp. failure and increased risk of lung cancer

Question 30

Bloods found in IPF

Answer 30

ANA 30% +ve Rheumatoid factor in 10% Immunoglobulins raised

Question 31

Bronchoalveolar lavage with IPF

Answer 31

Neutrophils and lymphocytes

Question 32

CXR in IPF

Answer 32

Decreased lung volume ground glass Bilateral lower zone reticulonodular shadows Honeycomb lung

Question 33

Management of IPF

Answer 33

O2 pulmonary rehab, opiates and palliative care Not high-dose steroids Trial 3-6months of azathioprine, oral glucocorticoids and high-dose acetylcysteine

Question 34

What is coal workers pneumoconiosis

Answer 34

Dust disease due to inhalation of coal particles in mines over 15-20 years Ingested by macrophages which die and release enzymes causing fibrosis Asymptomatic but can get bronchitis CXR - opacities - especially in upper zone Usually progressive onset of SOB and dry cough - can get black sputum, melanoptysis

Question 35

What is progressive massive fibrosis

Answer 35

Progression of CWP - progressive dyspnoea, fibrosis and eventually cor pulmonale CXR - upper zone fibrotic masses

Question 36

What is Caplan's syndrome?

Answer 36

Association of RA, pneumoconiosis and pulmonary rheumatoid nodules

Question 37

What is silicosis

Answer 37

Caused by inhalation of silica particles Very fibrogenic Progressive dyspnoea Upper and middle zone nodular pattern

Question 38

What is asbestosis

Answer 38

Inhalation of asbestos fibres (blue worse than white) causing fibrosis Progressive dyspnoea, clubbing, fine end-inspiratory crackles Increased risk of bronchial adenocarcinoma and mesothelioma

Question 39

What is malignant mesothelioma

Answer 39

Tumour of mesothelial cells in pleura Complex relationship with asbestos exposure - not all have pulmonary asbestosis Chest pain, dyspnoea, weight loss, clubbing, recurrent pleural effusions Metastases

Question 40

Management of malignant mesothelioma

Answer 40

Pemetrexed+cisplatin chemotherapy | Pleurodesis or indwelling drain may help