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Flashcards in ILD, EAA, IPF Deck (40)
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1

What is interstitial lung disease

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

2

What are the clinical features of ILD

Dyspnoea on exertion
Non-productive paroxysmal dry cough

Abnormal breath sounds

Chest pain - increased risk of pneumothorax due to fibrosis

Clubbing

3

Pathological features of ILD

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

4

3 categories of ILD

Known Cause
Associated with systemic disease
Idiopathic

5

Idiopathic causes of ILD

Idiopathic pulmonary fibrosis
Cryptogenic organising pneumonia
Lymphocytic interstitial pneumonia

6

ILD associated with systemic disorders

Sarcoidosis
RA
SLE
UC
Connective tissue disease
Autoimmune thyroid disease

7

Known causes of ILD

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

8

ILD CXR

Patchy shadowing

9

HR CT ILD

Fibrosis
Honeycombing - IPF

10

What is Extrinsic Allergic Alveolitis

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

11

What happens in acute and chronic phase of EAA?

Acute - alveoli infiltrated with acute inflammatory cells

Chronic - granuloma formation and obliterative bronchiolitis

12

Causes of EAA - the sensitizing thing

Bird fanciers and pigeon fanciers lung - proteins in bird droppings

Farmers and mushroom workers lung

Malt workers lung

Sugar workers lung

13

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

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

14

Chronic features of EAA

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

15

CXR in acute and chronic EAA

ACUTE
Upper-zone mottling/consolidation

CHRONIC
upper lobe fibrosis
Honeycomb lung

CT
Ground glass appearance

Bronchoalveolar lavage - CD8 T-lymphocytes and mast cells

16

Lung function tests in EAA

Restrictive - therefore FVC decrease more than FEV1
Decreased total lung capacity

17

Management of EAA acutely

Remove allergen and give O2 - acutely

Then oral prednisolone

18

Long term managment of EAA

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?!?

19

Causes of upper zone fibrotic shadowing on CXR

TB
EAA
Ankylosing spondylitis
Radiotherapy
Sarcoid

20

Causes of middle zone fibrotic shadowing on CXR

Progressive massive fibrosis

21

Causes of lower zone shadowing on CXR

IPF
Asbestosis

22

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

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

23

What is allergic bronchopulmonary aspergillosis?

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

24

Treatment of allergic bronchopulmonary aspergillosis ABPA

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

25

What is aspergilloma?

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

26

What is invasive aspergillosis?

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

27

What is Idiopathic Pulmonary Fibrosis?

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

28

Signs of IPF

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

29

Complications associated with IPF

Resp. failure and increased risk of lung cancer

30

Bloods found in IPF

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