Restrictive Thoracic Disease

Question 1

What is restrictive thoracic disease due to

Answer 1

disease within lungs ie DPLD

Question 2

What is the pathophysiology of restrictive thoracic disease

Answer 2

impaired alveolar gas exchange
alveolar barrier to O2 exchange
CO2 exchange unimpaired as alveolar ventilation normal
lower PaO2 normal PaCO2

Question 3

DPLD cause

Answer 3

fluid in alveolar air spaces

Question 4

cardiac Po oedema due to

Answer 4

raised Po venous pressure

Question 5

non cardiac Po oedema due to

Answer 5

normal Po venous pressure with leaky Po capillaries
sepsis or trauma
altitude sickness

Question 6

consolidation of alveolar air spaces

Answer 6

infective pneumonia(viral, bacterial, fungal, parasites)
infraction (pulmonary emoli/ vasculitis)
other causes (rheumatoid disease, drugs, cryptogenic)

Question 7

aetiology of DPLD

Answer 7

inflam - infiltrate of alveolar walls

Question 8

list extrinsic allergic alveolitis

Answer 8

farmers lung

avian

Question 9

list sarcoisosis

Answer 9

multi-system disease
lymphadenopathy
erythmea nodosum/ uveitis/ myocoraditis/ neuropathy

Question 10

fribrogenic DPLD

Answer 10

asbestosis

silicosis

Question 11

non fibrogenic DPLD

Answer 11

siderosis
stanosis
baritosis

Question 12

DPLD clinical syndrome

Answer 12

breathlessness on exertion
cough but no wheeze
finger clubbing
inspiratory lung crackles
central cyanosis
pulmonary fibrosis (end stage response to chronic inflammation)

Question 13

Diagnosis of DLPD

Answer 13

History
-occupation, drugs, pets, arthritis

Reduced lung volime

• lower FEV1 lower FVC normal ratio >75%
• peak flow = normal

Reduced gas diffusion

Arterial oxygen desaturation (lower PaO2 lower SaO2)
@rest or on exercise

Antibodies, Avian, Fungal, Auto-antibodies

Serum ACE and Ca raised in Sarcoid

Bilateral diffuse alveolar infiltrates on chest X ray

Echocardiogram to exclude heart failure + to diagnose second Po hypertension

High resolution CT scan inflammatory ground glass vs fibrotic nodular component of alveolar infiltrates in IPF

Bronchoalveolar lavage or induced septum - toe xclude pneumocystis, TB, other infection

transbronchial or thoracospic lung biopsy-rarely indicated

Question 14

DPLD treatment

Answer 14

remover trigger factors

treat reversible alveolitis

NB-danger of secondary super-infection due to immune suppression

Question 15

1st line DPLD treatment

Answer 15

systemic steroids

oral prednisolone-ICS = not effective

Question 16

2nd line treatment

Answer 16

oral Azathioprine

Question 17

IPF treatment

Answer 17

anti-fibrotic agents; pirfenidone, nintendanib

Question 18

O2 given in DPLD if

Answer 18

hypoxic

Question 19

When is lung transplantation necessary for DPLD

Answer 19

last resort end stage lung disease