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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?

A

normal

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
Q

What is the prognosis of IPF?

A

Shortened lifespan after diagnosis

50% 5 year survival

26
Q

What is the patho of IPF?

A

complication dysregulated inflammation that is poorly understood

27
Q

How is the fibrosis distributed in IPF?

A
  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
Q

Why are there bibasal crackles in IPF?

A

damage to alveoli

29
Q

What is the classic patient like in IPF?

A

Older >65 males

Smoking and Fhx

30
Q

What are some patient with IPF diagnosed with?

A

Patient may have had previous suspected COPD etc usually a delayed diagnosis (years late)

31
Q

What does spirometry and gas transfer like in IPF?

A

restrictive with reduced gas transfer

32
Q

When is DLCO low?

A

goes down if alveolar pathology and reduced gas transfer

33
Q

Why do you do a HRCT?

A

to show IPF pattern of fibrosis (bi-basal sub-pleural fibrosis)

34
Q

For IPF how do you exclude any other causes of pulmonary fibrosis?

A
  • rule out all other ILDs (exposure history, ANA panel, rheumatoid factor, ACCP, myositis panel for collage vascular disease)
  • drugs (amiodarone, methotrexate)
35
Q

How do you continue monitoring for IPF?

A

lung function, 6 min walking SO2 and quality life

Z Year3 Resp Conditions (20 decks)

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