Interstitial and Occupational Lung Diseases

Question 1

What is sarcoidosis?

Answer 1

Granulomatous disease (type 4 hypersensitivity) of unknown cause characterised by non-caveating granulomas.

Question 2

Presentation of acute sarcoidosis?

Answer 2

Erythema nodosum (tenderness of shins)
Bilateral hilar lymphadenopathy (large lymph nodes on x-ray)
Arthritis
Uveitis (inflammation of uvea in eye), parotitis (inflammation of salivary glands)
Fever
Often goes away by itself

Question 3

Presentation of chronic sarcoidosis?

Answer 3

Lung infiltrates (alveolitis)
	 Skin infiltrations
	 Peripheral lymphadenopathy
	 Hypercalcaemia
	 Other organ involvement renal, myocardial, neurological, hepatitis, splenomegaly

Question 4

What would you have to exclude first before making the diagnosis of sarcoidosis?

Answer 4

TB, lymphoma, carcinoma and fungal infection

Question 5

What pattern will be seen in a pulmonary function test with someone with sarcoidosis?

Answer 5

Restrictive

Question 6

Treatment of Sarcoidosis?

Answer 6

• In acute there is usually no treatment
• Steroids will be given if vital organs affected usually prednisolone.
• Oral steroids are usually needed in chronic sarcoidosis. Immunosupression drugs e.g. azathioprine, methotrexate, anti-TNF therapy.
• Monitor CXR and pulmonary function for several years.
• Note: There are often relapses.

Question 7

What is Extrinsic Allergic Alveolitis/ Hypersensitivity Pneumonitis?

Answer 7

• Type III hypersensitivity (Immune complex deposition) reaction to antigen lymphocytic alveolitis. There is inflammation in the alveoli within in the lung caused by hypersensitivity to inhaled organic dusts.

Question 8

Presentation of acute Extrinsic Allergic Alveolitis/ Hypersensitivity Pneumonitis?

Answer 8

• Cough, breathless, fever, myalgia
• Classically symptoms occur several hours after acute exposure (flu-like illness)
• Signs: +/- pyrexia, crackles (no wheeze!), hypoxia
• CxR: widespread pulmonary infiltrates

Question 9

Treatment of acute Extrinsic Allergic Alveolitis/ Hypersensitivity Pneumonitis?

Answer 9

oxygen, steroids and antigen avoidance

Question 10

Presentation of chronic Extrinsic Allergic Alveolitis/ Hypersensitivity Pneumonitis

Answer 10

Progressive breathlessness and cough
May be cracking, clubbing unusual
CxR shows pulmonary fibrosis - most commonly in the upper zones
PFTs: restrictive defect (low FEV1 & FVC, high or normal ratio, low gas transfer - TLCO)

Question 11

Treatment of chronic Extrinsic Allergic Alveolitis/ Hypersensitivity Pneumonitis

Answer 11

Remove antigen exposure, oral steroids if breathless or low gas transfer.

Question 12

What is idiopathic pulmonary fibrosis?

Answer 12

Fibrosis of unknown cause

Question 13

What is presentation of idiopathic pulmonary fibrosis?

Answer 13

• Clinical presentation: progressive breathlessness (several years), dry cough
• OE: clubbing, bilateral fine inspiratory crackles
• Ix: restrictive defect on PFT’s - reduced FEV1 and FVC with normal or raised FEV1/FVC ratio, reduced lung volumes, low gas transfer
• CxR - bilateral infiltrates;
• CT scan - reticulonodular fibrotic shadowing, worse at the lung bases, and periphery. Traction bronchiectasis. Honey-combing cystic changes.

Question 14

Treatment of idiopathic pulmonary fibrosis?

Answer 14

Ultimately you need a lung transplant but many patients are too old for this
There are anti fibrotic drugs pirfenidone and nintedanib but these only slow progression of disease they do not reverse damage that has already been done. Oxygen given if the patient is hypoxic.

Question 15

What are the two types of Coal Worker Pneumoconiosis

Answer 15

Simple Pneumoconiosis

Complicated - Progressive Massive Fibrosis

Question 16

Describe silicosis

Answer 16

Fibrosis caused by inhalation of silica particles
Occurs mainly in upper lobes
silica is highly fibrogenic so need much less silica vs coal for disease to occur

Question 17

What diseases are related to asbestos and what is the level of exposure and time period?

Answer 17

Lung Cancer- heavy asbestos exposure
Mesothelioma- light asbestos exposure, cancer doesn’t arise until 20-40 yrs after exposure, extremely poor prognosis
Pleural plaques- light asbestos exposure, doesn’t cause too much of a restrictive defect
Asbestosis- heavy asbestos exposure, massive fibrosis in lower lobes, poor prognosis

Question 18

Describe the pathology of sarcoidosis

Answer 18

Non-caseating granulomas

Occasionally there will be apical scarring in the lungs (scarring is apical not basal)

Question 19

What is caplans syndrome?

Answer 19

Combination of pneumoconiosis and rheumatoid arthritis which cause nodules in the lungs

Question 20

Chronic hypersensitivity pneumonitis fibrosis is most common in the ….

Answer 20

upper zones

Question 21

What will be elevated in sarcoidosis?

Answer 21

ACE

Question 22

Coal workers pneumoconiosis affects the ___1____ lobes
Silicosis affects the ____2____ lobes
Asbestosis affects the ____3______ lobes
Idiopathic pulmonary fibrosis affects the ___4____ lobes

Answer 22

1) upper
2) upper
3) lower
4) lower

Question 23

Describe simple coal workers pneumoconiosis

Answer 23

simple deposition of coal in the airways with formations of some nodules which can be seen on XR, there is minimal functional loss so not many symptoms

Question 24

Describe complicated progressive massive fibrosis coal workers pneumoconiosis

Answer 24

Progressive Massive Fibrosis patients develop round fibrotic masses particularly in the upper lobes and these masses sometimes have necrotic centres, this results in severe disability with dyspnea

Question 25

What electrolyte abnormality can occur in sarcoidosis?

Answer 25

Hypercalacaemia