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Yr 3 resp > idiopathic pulmonary fibrosis > Flashcards

idiopathic pulmonary fibrosis Flashcards

1
Q

definition of idiopathic pulmonary fibrosis

A

type of idiopathic interstitial pneumonia

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2
Q

aetiology of idiopathic pulmonary fibrosis

A

inflammatory cell infiltrate and pul fibrosis of unknown cause

A possible theory is that an unidentified insult causes damage to the alveolar epithelium, endothelium, and basement membrane. Cigarette smoke, organic or metal dust, gastro-oesophageal reflux disease (GORD), diabetes, and infection have each been associated with IPF; however, the exact inciting exposure or exposures remain unknown

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3
Q

pathology of idiopathic pulmonary fibrosis

A

Fibrosis and remodelling of the interstitium; chronic inflammation; hyperplasia of type II epithelial cells or type II pneumocytes.

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4
Q

RF for idiopathic pulmonary fibrosis

A

increased age

male

FH

smoking

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5
Q

epidemiology of idiopathic pulmonary fibrosis

A

commonest cause of interstitial lung disease

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6
Q

sx of idiopathic pulmonary fibrosis

A

dry, paroxysmal cough

exertional dyspnoea

malaise

reduced weight

arthralgia

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7
Q

signs of idiopathic pulmonary fibrosis

A

cyanosis

finger clubbing

fine end-inspiratory crepitations

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8
Q

Ix for idiopathic pulmonary fibrosis

A

blood

CXR

CT

spiromatory

BAL

99TCm-DTPA scan: (diethylene-triamine-penta-acetic acid) May refl ect disease activity.

lung biopsy

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9
Q

blood for idiopathic pulmonary fibrosis

A

ABG - low ox, and if severe high CO2

raised CRP, Ig, ANA (30% +ve), rheumatoid factor (10% positive)

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10
Q

CXR and CT for idiopathic pul fibrosis

A

CXR

  • reduced lung vol
  • bilateral lower zone reticulonodular shadows
  • honeycomb lung - advanced disease

CT

  • similar changes to CXR
  • more sensitive and essential for diagnosis
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11
Q

spirometry for idiopathic pulmonary fibrosis

A

restrictive

reduced transfer factor

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12
Q

BAL for idiopathic pulmonary fibrosis

A

may indicate activity of alveolitis: high lymphocytes (good response/prognosis) or high neutrophils and eosinophils (poor response/prognosis)

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13
Q

lung biopsy for idiopathic pulmonary fibrosis

A

needed for diagnosis

The histological changes observed on biopsy are referred to as usual interstitial pneumonia (UIP).

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14
Q

mx of acute exacerbation of idiopathic pulmonary fibrosis

A
  1. admission
  2. high dose steroids - oral pred daily for 4 wks then taper
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15
Q

mx of chronic idiopathic pul fibrosis

A
  1. antifibrotic therapy - pirfenidone or nintedanib
  2. smoking cessation, pul rehab, oxygen
  3. PPI if gastro-oesophageal reflux
  4. lung transplant
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16
Q

complications of idiopathic pul fibrosis

A
  • pul HTN
  • lung cancer
  • GORD
  • pul infection
  • pneumothorax
  • DVT
  • acs

Yr 3 resp (19 decks)

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