Chronic Interstitial Lung Disease (ILD)

Question 1

What is the most common ILD?

Answer 1

Idiopathic Pulmonary Fibrosis

Question 2

Which group of people is pulmonary fibrosis commonly seen in?

Answer 2

Men over 60 who smoke

Question 3

What are risk factors for idiopathic pulmonary fibrosis?

Answer 3

Smoking
Occupational (eg dust)
Drugs (methotrexate)
Viruses (EBV, CMV)

Question 4

What does the pulmonary fibrosis lead to?

Answer 4

Type 1 respiratory failure

Question 5

What are symptoms of pulmonary fibrosis?

Answer 5

Exertional dyspnoea
Dry unproductive cough

Question 6

How is pulmonary fibrosis diagnosed?

Answer 6

Spirometry: restriction

FEV1:FVC is greater than 0.7 but low FVC, below 0.8

High resolution chest CT:
Ground glass lungs and traction bronchiectasis

Question 7

Bronchiectasis is i_____ d_____ of bronchi and bronchioles within areas of pulmonary fibrosis

Answer 7

irreversible dilatation

Question 8

What is treatment for pulmonary fibrosis?

Answer 8

Smoking cessation

Vaccines

Meds to reduce scarring worsening: Pirfenidone, Nintendinib

Surgery (lung transplant)

Question 9

P_____ consists of occupationally acquired ILDs

Answer 9

Pneumoconiosis

Question 10

Silicosis is caused by inhaling s_____ ____

Answer 10

silicon dioxide

Question 11

What does silicon dioxide in the lungs cause?

Answer 11

Causes inflammation and scarring
The particles reduce the lung’s ability to take in oxygen

Question 12

What is visible on a chest XR of a patient with silicosis?

Answer 12

Eggshell calcification at hilar lymph nodes

Question 13

What substance other than silicon dioxide can commonly cause pneumoconiosis?

Answer 13

Asbestos

Question 14

What does inhalation of asbestos cause?

Answer 14

Scarring and thickening of the pleura.
Increases risk of mesothelioma and Type 1 respiratory failure

Question 15

Sarcoidosis is an idiopathic g______ disesae

Answer 15

granulomatous

Question 16

Who typically is affected by sarcoidosis?

Answer 16

20-40 y/o Afrocaribbean women

Question 17

What are symptoms of sarcoidosis?

Answer 17

Fever
Fatigue
Dry cough
Dyspnoea
Uveitis
Lupus pernio (blue-red nodules on nose and cheeks)

Question 18

Sarcoidosis is a chronic disease of unknown cause characterised the by the enlargement of l____ n____ in many parts of the body and widespread appearance of g____ derived from the reticuloendothelial system

Answer 18

lymph nodes
granulomas

Question 19

How is sarcoidosis diagnosed?

Answer 19

Chest XR showing bilateral hilar adenopathy (swollen glands) and pulmonary infiltrates

Diagnostic = biopsy

Question 20

What does a biopsy of sarcoidotic tissue show?

Answer 20

Non caseating granuloma

Question 21

What increases with presence of granulomas?

Answer 21

Serum calcium and serum ACE

Question 22

What is the treatment for sarcoidosis?

Answer 22

Corticosteroids

Question 23

Sarcoidosis is staged 1-_

Answer 23

4

Question 24

Lofgren syndrome is a specific acute clinical presentation of s____ s____

Answer 24

systemic sarcoidosis

Question 25

What does the classic triad of Lofgren syndrome consist of?

Answer 25

Fever Erythema nodosum (tender red bumps often symmetrical on shins) Bilateral hilar adenopathy

Question 26

What type of hypersensitivity reaction is hypersensitivity pneumonitis?

Answer 26

Type 3 (immune antibody-antigen complex deposition at lung tissue)

Question 27

What are risk factors of hypersensitivity pneumonitis?

Answer 27

Occupation eg farming Bird-keeping

Question 28

Name some types of Hypersensitivity Pneumonitis

Answer 28

Farmer's lung (most common, from mouldy hay) Pigeon fancier's lung from bird droppings Malt worker's lung Cheese worker's lung Humidifier fever

Question 29

What is the treatment for hypersensitivity pneumonitis?

Answer 29

Remove the allergen

Question 30

What type of hypersensitivity reaction is Goodpasture's syndrome?

Answer 30

Type 2

Question 31

What type of antibodies are made in Goodpasture's syndrome and what do they attack?

Answer 31

Anti-GBM (glomerular basement membrane) Attacks lungs and kidneys

Question 32

What does Goodpasture's cause to happen in the lungs and kidneys?

Answer 32

Lung fibrosis Glomerulonephritis

Question 33

How is Goodpasture's diagnosed?

Answer 33

Lung and kidney biopsy showing damage and immunoglobulin deposition Serology: Anti-GBM positive

Question 34

How is Goodpasture's treated?

Answer 34

Supportive Corticosteroids Plasma exchange to get rid of Anti-GBM

Question 35

What is Wegener's Granulomatosis also called?

Answer 35

Granulomatosis with polyangiitis (GPA)

Question 36

Wegener's Granulomatosis is the presence of both granulomatosis and ______

Answer 36

polyangiitis

Question 37

What is polyangiitis?

Answer 37

Vasculitis affecting small and medium vessels. Unknown cause, no genetic link. Typically causes ENT, lung and kidney symptoms (ELK)

Question 38

Granulomatosis with polyangiitis (GPA also called Wegener's Granulomatosis) can lead to inflamed, narrowed blood vessels and inflammatory tissue masses (granulomas). What is the effect of the narrow blood vessels and granulomas?

Answer 38

Narrow blood vessels means reduced blood flow and oxygen reaching body's tissues. Granulomas can destroy normal tissue

Question 39

Wegener's granulomatosis is a _-____ associated vasculitis

Answer 39

c-ANCA (antineutrophilic cytoplasmic antibody)

Question 40

What are symptoms of Wegener's granulomatosis?

Answer 40

(Think ELK: ENT, lungs, kidneys) ENT: saddle-shape nose, ear infection Lungs: diffuse alveolar haemorrhage and haemoptysis Kidneys: glomerulonephritis and haematuria

Question 41

How is Wegener's granulomatosis diagnosed?

Answer 41

ANCA positive However so is MPA (microscopic polyangiitis) and EGPA (Eosinophilic granulomatosis with polyangiitis)

Question 42

What is treatment for Wegener's granulomatosis?

Answer 42

Corticosteroids Immunosuppression eg Rituximab