Interstitial lung disease

Question 1

What does pulmonary fibrosis mean

Answer 1

Describes a group of diseases which produce interstitial lung damage and ultimately fibrosis and loss of the elasticity of the lungs

Question 2

Types of lung fibrosis

Answer 2

Replacement fibrosis secondary to lung damage

Focal fibrosis in response to irritants

Diffuse parenchymal lung disease

Question 3

Causes of replacement lung fibrosis

Answer 3

Infarction
TB
Pneumonia

Question 4

Irritants which can cause focal lung fibrosis

Answer 4

Coal dust and silica

Question 5

Which conditions are often associated with pulmonary fibrosis

Answer 5

Connective tissue diseases, including rheumatoid arthritis, SLE, systemic sclerosis and sjogren’s syndrome

Question 6

Which medications may be associated with lung fibrosis

Answer 6

Amiodarone
Nitrofurantoin
Bleomycin

Question 7

Which condition often co-exists with idiopathic pulmonary fibrosis

Answer 7

Chronic obstructive pulmonary disease

Question 8

Risk factors for pulmonary fibrosis

Answer 8

Smoking 
Certain occupations - exposure to asbestos
Environmental factors 
Chronic viral infections 
GORD with micro-aspiration

Question 9

Examples of environmental factors thought to contribute to pulmonary fibrosis

Answer 9

Pigeon breeding and contaminated ventilation systems

Question 10

Chronic viral infections which are thought to be risk factors for pulmonary fibrosis

Answer 10

Hepatitis C

Epstein-Barr viruses

Question 11

Presentation of pulmonary fibrosis

Answer 11

Age over 45 years 
Persistent breathlessness on exertion 
Persistent dry cough 
Fine bilateral inspiratory crackles on auscultation of the chest 
Clubbing 
Systemically unwell

Question 12

What signs might you find in an individual with interstitial lung disease on examination

Answer 12

Tachypnoea
Cyanosis
Signs of cor pulmonale and right heart failure in later stages

Question 13

Diagnosis of idiopathic pulmonary fibrosis

Answer 13

Clinical history and exam
Lung function testing(spirometry and gas transfer)
CXR
High resolution CT scan is gold standard

Question 14

Secondary investigations of diagnosis of pulmonary fibrosis cannot be made on initial ix

Answer 14

Bronchoalveolar lavage
Trasbronchial biopsy
Surgical lung biopsy

Question 15

How often should an individual with pulmonary fibrosis be assessed

Answer 15

At diagnosis
6 months
12 months
Repeat the lung function tests at shorter intervals if there is concern

Question 16

Supportive management of pulmonary fibrosis

Answer 16

Supportive therapy with oxygen for significant resting hypoxaemia

Pulmonary rehabilitation

Regular exercise and weight control

Vaccinations

Smoking cessation

PPI therapy

Question 17

Medical management of pulmonary fibrosis

Answer 17

Pirefenidone

Nintedanib

Question 18

When is pirfenidone recommended in management of pulmonary fibrosis

Answer 18

Forced vital capacity between 50% and 80% predicted

Treatment is stooped if there is evidence of disease progression

Question 19

Surgical management of pulmonary fibrosis

Answer 19

Lung transplant
Considered if a 10% or greater reduction in FVC over six months and a decrease in oxygen stats to below 89% or need for oxygen at rest

Question 20

Complications of pulmonary fibrosis

Answer 20

Pulmonary hypertension 
Lung cancer 
Pulmonary embolism 
Right ventricular heart failure 
Coronary heart disease

Question 21

Which medications can be used in management of cough if it is debilitating

Answer 21

Opioids

Discuss treatment with thalidomide with specialist if cough in interstitial lung disease is intractable

Question 22

Spirometry findings in idiopathic pulmonary fibrosis

Answer 22

a restrictive picture (FEV1 normal/decreased, FVC decreased, FEV1/FVC increased)

Impaired gas exchange - reduced transfer factor(TLCO)

Question 23

Causes of fibrosis affecting mainly upper zones

Answer 23

C - Coal worker's pneumoconiosis
H - Histiocytosis/ hypersensitivity pneumonitis
A - Ankylosing spondylitis
R - Radiation
T - Tuberculosis
S - Silicosis/sarcoidosis

Question 24

Causes of fibrosis mainly affecting lower zones

Answer 24

idiopathic pulmonary fibrosis
most connective tissue disorders (except ankylosing spondylitis) e.g. SLE
drug-induced: amiodarone, bleomycin, methotrexate
asbestosis