Restrictve Lung Disease.

Question 1

What can external factors causing thoracic restriction result in?

Answer 1

Chronic alveolar under ventilation with low PaO2, sats and lung volumes and raised PaCO2.

Question 2

What is DPLD?

Answer 2

Diffuse parenchymal lung disease.

Question 3

What classification system do we use for DPLD?

Answer 3

BTS classification.

Question 4

What factors outwith the lungs can cause restriction?

Answer 4

Skeletal e.g. Kyphosis.
Muscle weakness due to trauma, neuro etc.
Abdominal obesity or ascites.

Question 5

What does chronic thoracic restriction due to causes out with the lungs lead to in terms of blood gasses?

Answer 5

Under ventilation with a low PaO2 and high PCO2 and reduced lung volumes.

Question 6

What does restrictive thoracic disease due to disease of the alveolar structures lead to in terms of blood gasses?
Why?

Answer 6

Decreased PaO2 and decreased SaO2 due to decreased O2 exchange because there is alveolar/arteriolar disease.
There are normal levels of PCO2 as CO2 is very soluble and there is still normal alveolar em ventilation and so it is blown off.

Question 7

What are the different mechanisms of disease that cause DPLD?

Answer 7

Fluid in the alveolar space.
Consolidation of alveolar air spaces
Inflammatory infiltrate in the alveolar walls.

Question 8

What is BOOP and what is it otherwise known as?

Answer 8

Bronchiolitis obliterans organising pneumonia.

Also known as COP - crypto genie organising pneumonia.

Question 9

What is inflammatory infiltrate in the alveolar walls called?

Answer 9

Alveolitis.

Question 10

What can cause alveolitis?

Answer 10

Graulomatous alveolitis, extrinsic allergic alveolitis, fibrosing alveolitis, sarcoidosis, can also be drugs, fumes or autoimmune.

Question 11

What are two types of fibrogenic pneumoconiosis?

Answer 11

Asbestosis and silicosis.

Question 12

What are three types of nonfibrogenic pneumoconiosis and what causes them?

Answer 12

Siderosis (iron), stanosis (tin) and baritosis from barium.

Question 13

What signs do we find on examination of DPLD?

Answer 13

Breathlessness on exertion, cough but no wheeze, finger clubbing, inspiratory crackles, Central cyanosis and pulmonary fibrosis occurs at the end stage.

Question 14

What lung volumes do we get with DPLD?

Answer 14

Reduced FEV1 and FVC 1 with a normal ratio of over 75%

Normal peak flow.

Question 15

What blood results would we expect to be raised in sarcoidosis?

Answer 15

Serum ACE and Ca.

Question 16

What are the pharmacological and non pharmacological treatments of DPLD?

Answer 16

Remove any allergic trigger.
Treat any inflammation I.e. If see ground glass on high resolution CT scan.
O2 if hypoxaemia
Lung transplant for end stage disease.

1st line treatment - systemic corticosteroids I.e. Oral prednisolone. Inhaled are not affective.

2nd line treatment - oral azathioprine (steroid sparing)
Anti fibrotic agents - Pirfenidone
Anti oxidant agents - acetlycysteine

Question 17

What can cause drug induced alveolitis?

Answer 17

Amiodarone, bleomycin, methotrexate or gold.