ILDs

Question 1

Describe the epidemiology of asbestos-related lung disease

Answer 1

Increasing prevalence recently
Commoner among certain professions eg. builders, ship builders
Almost entirely male predominance

Question 2

Describe the types of asbestos-related lung disease

Answer 2

Pleural plaques: non-malignant thickening of the pleura
Asbestosis: pulmonary fibrosis due to asbestos
Mesothelioma: malignant disease of the pleura

Question 3

Describe the presentation of asbestos-related lung disease

Answer 3

Pleural plaques: may be incidental finding on CXR/HRCT
Asbestosis: presents with progressive SOB, chronic dry cough, wheeze
Mesothelioma: pleuritic CP, SOB, cough, fatigue, weight loss

Question 4

Describe the signs of asbestos-related lung disease on examination

Answer 4

Asbestosis: signs consistent w fibrosis

• Clubbing
• Fine late inspiratory crackles in bases

Mesothelioma: usually presents w pleural effusion
-Unilat absent breath sounds and dullness to percussion

Question 5

Describe the investigations for asbestos-related lung disease

Answer 5

• History and examination: focus on occupational exposure
• Bloods: FBC, CRP, U+Es, LFTs, BNP
• CXR (pleural thickening, linear interstitial fibrosis)
• HRCT diagnostic
• Spirometry

Question 6

Describe the management of asbestos-related lung disease

Answer 6

No treatment
Asbestosis:
-Conservative: no smoking, exercise, vaccinations, pulmonary rehab
-Medical: O2 therapy

Mesothelioma:

• Medical: chemorad
• Surgical: pneumonectomy, pleurectomy

Question 7

Describe the prognosis of asbestos-related lung disease

Answer 7

Pleural plaques- usually fine, do not progress
Asbestosis- prognosis related to the extent of fibrosis
Mesothelioma- highly lethal. 90-95% die within 5 years

Question 8

Describe the epidemiology of idiopathic pulmonary fibrosis

Answer 8

Affects older adults
M > F
No geographic/racial predilection

Question 9

Define IPF

Answer 9

IPF is a chronic progressive lung disease with scarring and fibrosis that occurs without a known cause

Question 10

Describe the presentation of IPF

Answer 10

Main symptom: progressive SOB

• Chronic dry cough
• Fatigue
• Weight loss

Question 11

Describe the signs of IPF on examination

Answer 11

General: dyspnoeic, thin
Hands: clubbing
Chest: fine late inspiratory crackles at the lung bases

Question 12

Describe the differential diagnoses for IPF and how these are explored through history/exam/Ix

Answer 12

Respiratory:

• Other interstitial lung disease: history for occupational exposures and other symptoms suggesting connective tissue disease/etc, exam for other signs of systemic disease
• COPD: smoking history likely to be more, spirometry shows obstructive picture

Cardiac:
-HF: history for other symptoms/RFs, exam for signs of oedema/raised JVP, BNP and echo

Question 13

Describe the investigations for IPF/pulmonary fibrosis

Answer 13

• History and examination
• Bloods: FBC, CRP, U+Es, LFTs, BNP, antibodies
• CXR
• HRCT: fine reticular (ground-glass) opacities with honey-combing
• Spirometry: restrictive picture (FEV1/FVC >0.7)
• Lung biopsy (not needed for all)

Question 14

Describe the management of IPF

Answer 14

Conservative:

• Exercise and healthy lifestyle, no smoking
• Vaccinations
• Pulmonary rehab if needed

Medical:

• Specific drugs
• Treat cause eg. DMARDs/biologics for systemic disease
• Oxygen therapy

Surgical:
-Lung transplantation

Question 15

Define pneumoconiosis

Answer 15

Pneumoconioses are a group of interstitial lung diseases caused by exposure to inorganic/mineral dust eg. silicon, coal dust

Question 16

Describe the epidemiology of pneumoconioses

Answer 16

Usually associated with occupational exposure
M > F
Not very common

Question 17

Describe the presentation of pneumoconioses

Answer 17

Most commonly progressive dyspnoea
+ chronic dry cough, wheeze
+fatigue, weight loss

Question 18

Describe the investigations for pneumoconioses

Answer 18

Same as for other ILDs

• Bloods to rule out systemic disease, HF
• CXR first (upper lobe nodular opacities, egg-shell calcification of the hilum in silicosis)
• HRCT diagnostic (ground glass, honeycombing in upper lobes)
• Spirometry (commonly obstructive in coal + silicosis), used for prognosis + treatment planning

Question 19

Describe the management of pneumoconioses

Answer 19

Similar to IPF:
Conservative: eg. pulmonary rehab, lifestyle, vax
Medical: bronchodilators, oxygen therapy
Surgical: transplant

Question 20

Define extrinsic allergic alveolitis. What is the new term?

Answer 20

Now called hypersensitivity pneumonitis

A group of lung diseases caused by exposure to organic particles eg. mould, dust

Question 21

What are some types of EAA/hypersensitivity pneumonitis?

Answer 21

Malt-workers lung: Aspergillus clavatus + fumigatus
Pigeon fanciers lung: pigeon faeces proteins
Farmer’s lung: Actinomycetes
Drug induced HP

Question 22

Describe the pathophysiology of EAA/HP

Answer 22

Exposure to organic dust (non-human protein) causes an immune reaction -> inflammation of the airways

Question 23

Describe the presentation of EAA/HP

Answer 23

3 syndromes:
Acute (hours after exposure):
-Fever and chills, myalgia
-Dry cough and SOB

Sub-acute (weeks-months):

• SOB and productive cough
• May not have a temporal relationship to exposure if constantly around eg. mould in the house

Chronic (months-years):

• SOB, cough
• Fatigue, weight loss

Question 24

Describe the investigations for EAA/HP

Answer 24

Bloods: FBC, CRP, ESR, U+Es, serology 
CXR: patchy, nodular infiltrates
HRCT if needed: reticulonodular opacities
Spirometry : may be either R or O
BAL/biopsy

Question 25

Describe the management of EAA/HP

Answer 25

Conservative:
-Avoidance of the trigger

Medical:
-Steroids for acute/sub-acute