Fibrotic and Idiopathic Interstitial Lung Disease

Question 1

Give 5 conditions that cause predominately upper zone fibrosis

Answer 1

• hypersensitivity pneumonitis
• sarcoidosis
• ankylosing spondylitis
• radiation-induced pulmonary fibrosis
• tuberculosis

Question 2

Give 4 causes of predominately lower zone fibrosis

Answer 2

• idiopathic pulmonary fibrosis
• most connective tissue disorders e.g. SLE
• drug-induced: amiodarone, bleomycin, methotrexate
• asbestosis

Question 3

What is idiopathic pulmonary fibrosis

Answer 3

a chronic lung condition characterised by progressive fibrosis of the lungs with no apparent cause

Question 4

Risk factors for idiopathic pulmonary fibrosis

Answer 4

• older age (60-70 years)
• male
• FHx

Question 5

Features of idiopathic pulmonary fibrosis

Answer 5

• progressive exertional dyspnoea
• fine end-inspiratory fine bi-basal fine crackles on auscultation
• dry cough
• clubbing

Question 6

What are the typical findings in spirometry for idiopathic pulmonary fibrosis?

Answer 6

Restrictive pattern:
* FEV1 normal or decreased
* FVC decreased
* FEV1/FVC increased

Question 7

What is the typical imaging finding for idiopathic pulmonary fibrosis?

Answer 7

• Chest x-ray: peripheral, Bilateral, asymmetrical, reticular opacities (ground-glass)
• High-resolution CT chest (GS) - increased reticulation, honeycombing

Question 8

What is the management for idiopathic pulmonary fibrosis?

Answer 8

• acute - admission, supportive care, prednisolone
• Antifibrotic agent: pirfenidone or nintedanib
• pulmonary rehabilitation

Question 9

What is sarcoidosis?

Answer 9

A chronic multisystem disorder of unknown aetiology characterised by non-caseating granulomas

Question 10

What are granulomas

Answer 10

inflammatory nodules full of macrophages

Question 11

Describe the epidemiology of sarcoidosis

Answer 11

• 20-40 year old
• women
• black ethnic origin

Question 12

What is the most commonly affected organ in sarcoidosis

Answer 12

lungs

Question 13

How are the lungs affected by sarcoidosis

Answer 13

• patients may present with dry cough and gradual dyspnoea
• bilateral hilar lymphadenopathy
• pulmonary nodules
• pulmonary fibrosis

Question 14

Skin manifestations of sarcoidosis

Answer 14

• erythema nodosum - tender, red nodules on shins
• lupus pernio - raised purple plaques on nose, cheeks

Question 15

Systemic features of sarcoidosis

Answer 15

• fever
• fatigue
• weight loss

Question 16

Ocular symptoms of sarcoidosis

Answer 16

• uveitis
• conjunctivitis

Question 17

What electrolyte change may be seen in sarcoidosis

Answer 17

hypercalcaemia

Question 18

What is Lofgren’s syndrome

Answer 18

acute form of sarcoidosis characterised by:
* bilateral hilar lymphadenopathy
* erythema nodosum
* polyarthralgia

Question 19

What blood tests are done to investigate sarcoidosis

Answer 19

• serum angiotensin-converting enzyme (ACE) - raised
• calcium - raised
• ESR - raised

Question 20

What imaging is involved in the diagnosis of sarcoidosis

Answer 20

• CXR - lymphadenopathy, interstitial infiltrates, fibrosis
• MRI - identify areas of sarcoidosis involvement

Question 21

What would histology of sarcoidosis characteristically show

Answer 21

non-caseating granulomas with epithelioid cells

Question 22

How is sarcoidosis managed

Answer 22

• asymptomatic - conservative
• symptomatic: 1st - steroids, 2nd - methotrexate

Question 23

What is the prognosis for sarcoidosis

Answer 23

• spontaneously resolves in around 50% of patients with stage I/II disease
• can progress to pulmonary fibrosis in some patients

Question 24

Give 3 factors associated with a poor prognosis for sarcoidosis

Answer 24

• insidious onset, symptoms > 6 months
• extrapulmonary manifestations: e.g. lupus pernio, splenomegaly
• CXR: stage III-IV features