Interstitial lung diseases

Question 1

What are ILDs?

Answer 1

Term to describe a number of conditions that primarily affect lung parenchyma in a diffuse manner

Question 2

General clinical features of ILDs

Answer 2

dyspnea on exertion, non-productive paroxysmal cough, abnormal breath sounds + CRX, restrictive pulmonary spirometry

Question 3

General pathology of ILDs

Answer 3

fibrosis + remodelling of interstitial, chronic inflammation, hyperplasia of type II pneumocytes

Question 4

How can ILDs be broadly classified?

Answer 4

1. those with known cause e.g. occupational, hypersensitivity reactions (extrinsic allergic alveolitis)
2. those associated with systemic disorders e.g. sarcoidosis, RA, SLE
3. idiopathic

Question 5

What are the following a cause of?
Bird-fanciers and pigeon-fanciers lung
Farmer's and mushroom workers lung
malt worker;s
bagassosis or sugar workers?

Answer 5

extrinsic allergic alveolitis

Question 6

In extrinsic allergic alveoli’s, what does inhalation of allergens provoke in sensitive individuals?

Answer 6

a type 3 hypersensitivity reaction

Question 7

Acute presentation of extrinsic allergic alveolitis

Answer 7

fevers, riggers, myalgia, dry cough, dyspnea, crackles

Question 8

Chronic presentation of extrinsic allergic alveolitis

Answer 8

increasing dyspnea, weight loss, type 1 resp failure

cor pulmonae

Question 9

What would CXR for extrinsic allergic alveolitis show?

Answer 9

Acute: upper-zone mottling/consolidation

Chronic: upper zone fibrosis, honeycomb lung

Question 10

What would blood tests for extrinsic allergic alveolitis show?

Answer 10

ESR up

Positive serum precipitins

Question 11

What would lung function tests show for extrinsic allergic alveolitis?

Answer 11

Reversible restrictive defect

Question 12

Acute Mx for extrinsic allergic alveolitis

Answer 12

Remove allergen
Give O2
Oral prednisolone

Question 13

Chronic Mx for extrinsic allergic alveolitis

Answer 13

avoid exposure
long term steroids
compensation?

Question 14

What’s sarcoidosis?

Answer 14

multisystem granulomatous disorder of unknown cause

Question 15

Presentation of acute sarcoidosis

Answer 15

erythema nodosum (red skin lumps)
polyarthralgia (aches in the joints)

Pulm Sx: dry cough, progressive dyspnea, decreased exercise tolerance + chest pain

Question 16

What would blood test show for sarcoidosis?

Answer 16

Raised ESR, lymphopenia, raised LFT, serum ACE raised,

Question 17

What does tissue biopsy show in sarcoidosis?

Answer 17

non-caseating granulatomata

Question 18

what is punched out lesions in terminal phalanges a feature of?

Answer 18

sarcoidosis

Question 19

What do 90% of those with sarcoidosis have on their CRX?

Answer 19

abnormal CXR with bilateral hilar lymphadenopathy (BHL) +/- pulm infiltrates or fibrosis

Question 20

Mx sarcoidosis

Answer 20

Bed rest + NSAIDs for acute

Corticosteroids if more serious (parenchymal lung disease, uveitis, hypercalcaemia, neuro or cardiac involvement)

Question 21

What is idiopathic pulmonary fibrosis (IPF) also known as?

Answer 21

crytogenic fibrosing alveolitis

Question 22

whats the most common cause of interstitial lung disease?

Answer 22

idiopathic pulmonary fibrosis

Question 23

in IPF, what does progressive fibrosis lead to?

Answer 23

it limits pt’s ability to breath –> terminal chest infection + hypoxic damage

Question 24

Presentation of IPF

Answer 24

dry cough, exertion dyspnea, malaise, weight loss, arthralgia

signs: cyanosis, finger clubbing, fine end-inspiratory crepitations

Question 25

Blood results for IPF

Answer 25

ABG (PaO2 down, PaCO2 raised if severe)
CRP raised
immunoglobulins raised

Question 26

What is usual interstitial pneumonia?

Answer 26

The histological variable pattern of inflammation + fibrosis you see in IPF

Question 27

Mx for IPF

Answer 27

best supportive care
DO NOT use high dose steroids

? clinical trials for lung transplant

Question 28

Secondary causes of ILD

Answer 28

RA
Asbestos exposure (may --> malignant mesothelioma and lung cancer)

Both seem to show the pattern of usual interstitial pneumonia