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Z Year3 Resp Conditions > Fibrotic Lung Disease > Flashcards

Fibrotic Lung Disease Flashcards

1
Q

What are two types of fibrotic lung disease?

A
  • Idiopathic pulmonary fibrosis (IPF)

- Cystic fibrosis

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2
Q

What happens in IPF?

A

scar tissue formation within lungs

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3
Q

What are RF for IPF?

A
  1. FHx
  2. Cigarette smoking
  3. Advanced age
  4. Male sex
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4
Q

What type of inheritance is CF?

A

autosomal recessive

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5
Q

What gene is affected in CF?

A

mutation on CFTR gene on chromosome 7

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6
Q

What are symptoms of CF?

A
  • cough, wheeze, recrrent infection

- pancreatic insufficiency, male infertility etc

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7
Q

What is needed for diagnosis of CF?

A
  1. Sweat test: sodium and chloride >60mmol/L

2. Genetic screen for mutation

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8
Q

What is the management of CF?

A
  1. chest physiotherapy
  2. antibiotics acute exacerbation
  3. mucolytics
  4. bronchodilator
  5. treat other issues e.g diabetes + give pancreatic enzymes
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9
Q

What is the median survival for CF?

A

41 years

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10
Q

What are the progressive symptoms of IPF?

A
  1. Exertional Dyspnoea
  2. Progressive SOB
  3. Dry Cough
  4. Weight loss, fatigue and malaise
  5. Slow insidious onset
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11
Q

What are some signs in IPF?

A
  1. Cyanosis
  2. Finger clubbing
  3. Find-end inspiratory crepitations – bi-basal fine end inspiratory crepitations
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12
Q

What are DDx for IPF?

A
  1. Non-specific interstitial pneumonia
  2. Cryptogenic organising pneumonia
  3. Acute interstitial pneumonia
  4. Asbestosis
  5. Sarcoidosis
  6. Etc
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13
Q

What imaging is done in IPF?

A
  1. CXR

2. High res CT

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14
Q

What does CXR show in IPF?

A
  1. basilar
  2. peripheral
  3. bilateral
  4. asymmetrical
  5. reticular opacities
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15
Q

What does high res CT show in IPF?

A
  • basilar- and subpleural-predominant areas of increased reticulation honeycombing
  • possible traction bronchiectasis or bronchiolectasis
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16
Q

What other tests can be done in IPF?

A
  1. Anti-nuclear antibodies
  2. Rheumatoid factor: normal or midly elevated
  3. Anti-cyclic citrullinated peptide: normal
  4. Mysositis panel: normal
  5. Pulmonary function test: restrictive
  6. Lung biopsy for diagnosis
17
Q

What are the ANA and RF usually in IPF?

A

normal or midly elevated

18
Q

What is aCCP and mysositis in IPF?

19
Q

What does pulmonary function tests show in IPF?

A

restrictive

20
Q

What may be needed for diagnosis?

A

lung biopsy

21
Q

What is the management of an acute exacerbation of IPF?

A

1st line: admission to hospital + high dose corticosteroid

-Cytotoxic therapy

22
Q

What if 1st line treatment of chronic IPF?

A
  1. antifibrotic therapy (e.g. pirfenidone 267mg x3 or nintedamib 130mg x2)
  2. smoking cessation
  3. pulmonary rehab
  4. oxygen
  5. PPI
23
Q

What is 2nd line treatment for IPF?

A

lung transplant

24
Q

What are possible complications of IPF?

A
  1. Pulmonary hypertension
  2. Lung cancer
  3. GORD
  4. Pulmonary infection
  5. Pneumothroax
  6. PE
25
What is the prognosis of IPF?
Shortened lifespan after diagnosis | 50% 5 year survival
26
What is the patho of IPF?
complication dysregulated inflammation that is poorly understood
27
How is the fibrosis distributed in IPF?
1. Fibrosis of the parenchyma starts around the pleura often basally in a “usual interstitial pneumonia” pattern 2. bibasal subpleural fibrosis and sometimes happen apically
28
Why are there bibasal crackles in IPF?
damage to alveoli
29
What is the classic patient like in IPF?
Older >65 males | Smoking and Fhx
30
What are some patient with IPF diagnosed with?
Patient may have had previous suspected COPD etc usually a delayed diagnosis (years late)
31
What does spirometry and gas transfer like in IPF?
restrictive with reduced gas transfer
32
When is DLCO low?
goes down if alveolar pathology and reduced gas transfer
33
Why do you do a HRCT?
to show IPF pattern of fibrosis (bi-basal sub-pleural fibrosis)
34
For IPF how do you exclude any other causes of pulmonary fibrosis?
- rule out all other ILDs (exposure history, ANA panel, rheumatoid factor, ACCP, myositis panel for collage vascular disease) - drugs (amiodarone, methotrexate)
35
How do you continue monitoring for IPF?
lung function, 6 min walking SO2 and quality life

Z Year3 Resp Conditions (20 decks)

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