Fibrotic Lung Disease Flashcards

1
Q

What is fibrotic lung disease?

A

A restrictive lung disease caused by fibrosis to the lungs making breathing very difficult.

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2
Q

Name diseases associated with upper zone fibrotic lung disease.

CHARTS !!!

A

C- Coal workers pneumoconisosis
H - Histiocytosis/hypersensitivity penumonitis
A - Ankylosing spondylitis
R - Radiation
T - TB
S - Silicosis/sarcoidosis

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3
Q

What is the most common condition in lower zone fibrotic lung disease?

ACIDS

A

Idiopathic pulmonary fibrosis (IPF)

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4
Q

What are the causes of lower zone pulomnary fibrosis?

AAAI

A

A - Asbestos
A - Amioderone
A - Autoimmune conditions (SLE, RA)
I - Idiopathic pulmonary fibrosis

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5
Q

Define idiopathic pulmonary fibrosis (IPF).

A

A restrictive chronic lung condition characterised by progressive fibrosis of the interstitium of the lungs - IPF occurs when there is NO underlying cause.

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6
Q

What is the typical demographic for idiopathic pulmonary fibrosis?

A

Common in ages 50-70, predominantly MALE.

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7
Q

What are the symptoms of idiopathic pulmonary fibrosis?

A
  • Progressive dyspnoea on exercise
  • Dry cough
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8
Q

What are the signs of idiopathic pulmonary fibrosis?

A

Auscultation: Bi-basal fine end-inspiratory crepitations
Clubbing.

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9
Q

What is the spirometry pattern in idiopathic pulmonary fibrosis?

A

Classically a restrictive pattern.

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10
Q

What are the typical spirometry values in idiopathic pulmonary fibrosis?

A
  • FEV1 - normal/decreased
  • FVC - decreased
  • FEV1/FVC - increased (above 0.7)
  • TLCO (imapired gas exchange) - REDUCED
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11
Q

What does TLCO measure?

A

Transfer factor of lung for carbon monoxide, indicating diffusion of gas across the alveolar blood membrane.

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12
Q

What imaging techniques are used for diagnosing fibrotic lung disease?

A
  • CXR for investigation
  • High-resolution CT for diagnosis
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13
Q

What are positive findings on imaging (CXR or CT) for idiopathic pulmonary fibrosis?

A
  • Bilateral interstitial shadowing
  • Ground glass
  • Honeycombing
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14
Q

What blood tests are relevant for idiopathic pulmonary fibrosis?

A
  • ANA positive in 30%
  • Rheumatoid factor + in 10%
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15
Q

What is the management strategy for idiopathic pulmonary fibrosis?

A
  • Pulmonary rehabilitation
  • Drugs: Pirfenidone (slows down disease progression)
  • Long-term: Supplementary O2 + lung transplant
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16
Q

What is the prognosis for idiopathic pulmonary fibrosis?

A

Poor life expectancy = 3-4 years.