Lecture 13: Interstitial Lung Disease

Question 1

what makes up the interstitium

Answer 1

• alveolar endothelium
• capillary endothelium
• basement membrane
• connective tissue

Question 2

what can the interstitium be thickened by

Answer 2

• fluid
• cells
• fibrosis

Question 3

what are functions of the extracellular matrix

Answer 3

• tensile strength / elasticity
• low resistance for effective gas exchange
• tissue repair / modelling

Question 4

what are the main types of interstitial lung diseases

Answer 4

• sarcoidosis
• connective tissue diseases
• hypersensitivity pneumonitis
• idiopathic pulmonary fibrosis

Question 5

presentation of idiopathic pulmonary fibrosis

Answer 5

• symptoms appear gradually
• slowly progressive exertional dyspnoea
• non-productive cough
• inspiratory crackles
• potential clubbing

Question 6

what can you see on a CT scan with idiopathic pulmonary fibrosis

Answer 6

• basal distribution
• subpleural
• traction bronchiectasis
• honeycombing

Question 7

which people does idiopathic pulmonary fibrosis tend to affect

Answer 7

• median age 66 years
• more common in men
• more likely to occur in smokers

Question 8

what is medication for idiopathic pulmonary fibrosis and their side effects

Answer 8

• pirfenidone
• nintedanib
• weight loss
• GI upset
• photosensitivity

Question 9

what is hypersensitivity pneumonitis

Answer 9

diffuse inflammation of parenchyma in response to inhaled antigen

Question 10

presentation of hypersensitivity pneumonitis

Answer 10

acute
- SOB, cough, fever, crackles
subacute
- gradual onset of symptoms with weight loss
chronic
- incomplete resolution with removal of antigen
- may lead to irreversible fibrosis

Question 11

how can you diagnose hypersensitivity pneumonitis

Answer 11

serum precipitins

Question 12

how can hypersentivity pneumonitis be managed

Answer 12

steroids

Question 13

which drugs are known to induce interstitial lung diseases

Answer 13

• nitrofurantonin
• amiodarone
• methotrexate

Question 14

histroy of connective tissue disease related ILDs

Answer 14

• dry eyes / mouth (sicca)
• raynaud’s
• joint pain / swelling
• rashes

Question 15

management of connective tissue disease related ILDs

Answer 15

• biologics
• steroids
• immunosuppression

Question 16

what do blood tests show in hypersensitive pneumonitis

Answer 16

• antinuclear antibodies

- rheumatoid factor

Question 17

what would a chest x ray show in case of ILD

Answer 17

reticular shadowing

Question 18

what would the FEV1/FVC ratio be like in a patient with ILD

Answer 18

normal or high

Question 19

what is sarcoidosis

Answer 19

multisystem granulomatous disorder

Question 20

who does sarcoidosis affect more

Answer 20

• 3x more common in afro-caribbeans

- 75% of cases are aged 30-60

Question 21

how do you diagnose sarcoidosis

Answer 21

• do biopsy

- look for non-necrotising / non-caseating granuloma

Question 22

presentations of sarcoidosis

Answer 22

• cough, SOB, wheeze
• anterior uveitis on eyes
• lymph node biopsy performed
• hypercalcaemia
• lofgren’s syndrome

Question 23

what is lofgren’s syndrome

Answer 23

• erythema nodosum
• bilateral hilar lymphadenopathy
• arthralgia

Question 24

systemic symptoms of pulmonary sarcoidosis

Answer 24

• fatigue
• weight loss
• fevers
• sweats
• arthralgia

Question 25

how can you diagnose sarcoidosis

Answer 25

combination of:

• clinical picture
• exclusion of alternate diagnoses
• biopsy of affected tissue

Question 26

what is treatment for sarcoidosis

Answer 26

• target organ that is affected if extra-thoracic
• corticosteroids for 6-24 months
• additional immunosuppressants may be necessary

Question 27

what are the results of a lung function test in interstital lung disease

Answer 27

FEV1/FVC ratio may be high, but could also be normal