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(6-10) Translational Medical Science > Trigger 6: IPF > Flashcards

Trigger 6: IPF Flashcards

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Biology 101 flashcards USMLE® Step 1 flashcards
1
Q

incidence of IPF

A

is increasing

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2
Q

idiopathic pulmonary fibrosis is

A

a type of interstitial lung disease

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3
Q

pulmonary fibrosis is the ..

A

end stage of a heterogeneous group of interstitial lung disease

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4
Q

which interstitial lung disease is the most common

A

IPF

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5
Q

how many deaths per year in the UK

A

> 5,000

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6
Q

how may patients in the EU with IPf

A

360,000

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7
Q

median suvrival

A

2-3 years

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8
Q

how many active IPF trial

A

> 200

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9
Q

most common age to get IPF

A

70-75

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10
Q

currently no treatments which

A

cure or reverse scarring

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11
Q

causes of IPF

A

occurs when alveoli get damaged and increasingly scarred

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12
Q

although idiopathic, IPF is linked to

A 
o	dust, wood or metal exposure
o	viral infection
o	family history of IPF
o	Gastro-oesophageal reflux
disease
o	smoking
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13
Q

pathological characterisation

A
  • excessive accumulation of extracellular matrix (ECM) and remodelling of the lung architecture, this causes:
    1) proliferation and apoptosis of fibroblasts
    2) accumulation and breakdown of ECM- altered mesenchymal cells
    3) Loss of normal type 1 alveolar epithelium and replacement by hyperplastic type II cells found in IPF patients
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14
Q

basic mechanism of IPF

A
  • Injury
  • Acute inflammatory responses
  • persistent irritant damaging agents
  • production of pro-inflammatory, angiogenic, and fibrogenic cytokines, as well as chemokines, growth factors and tissue disruptive enzymes
  • increased accumulation of extracellular matrix and fibrotic lesions
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15
Q

features of IPF

A
  • scarring- honeycombing
  • loss of elasticity
  • inflammation
  • loss of air filled spaces
  • reduction in surface area
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16
Q

thoracic imaging shows

A

pleural surface has a cobblestone appearance.

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17
Q

on cross section

A

cobblestone reatino show areas of airspace enlargement and fibrotic retraction

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18
Q

airspace enlargement and fibrotic retraction

A

honeycombing

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19
Q

symptoms of IPF

A
  • Shortness of breath
  • Coughing
  • Fever
  • Weight loss
20
Q

IPF onset

A

rapid

21
Q

physical examination

A
  • listen to the lungs

- looking at fingers

22
Q

what will the lungs sound like

A
  • lunk crackles ( IPF and asbestos lungs)

- pleural rub

23
Q

what do fingers look like

A

clubbed

24
Q

which pulmonary function tests can be done

A 
o	Forced vital capacity
o	Diffusion capacity of lung for CO (DLco)
o	FEV1/FVC ratio
o	Oxyhaemoglobin stas
o	6-minute walk test
25
Q

blood test

A

no specific blood test available

26
Q

why is it important that patients receive an accurate diagnosis

A

to ensure correct treatment and management decision

27
Q

diagnoses often invole

A

invasive and non invasive diagnostic procedures

28
Q

Non-invasive diagnostic procedure

A

secured by the presence of UIP (usual interstitial pneumonia)- using High resolution computed tomography (HRCT)

29
Q

what will HRCT show

A
  • reticular lines

- traction bronchiectasis

30
Q

evaluation of honeycombing shown on HRCT has been found vulnerable to

A

significant inter observer variability

31
Q

diagnosis can sometime be made just using

A

high resolution computed tomography (HRCT)

32
Q

name some invasive diagnostic procedure

A

bronchoscopy

bronchopleural laval (BAL)

lung biopsy

33
Q

BAL is

A

analysed for infection or malignancies

34
Q

10% of patients cannot undergo surgical biopsy due to

A

AE and advanced disease

35
Q

diagnosis pathway

A

1) patient with suspected ILD
2) Comprehensive history, physical exam and appropriate lab testing
3) Thoracic imaging (Chest-xray, HRCT)
4) intermediate diagnosis

36
Q

after intermediate diagnosis what question is asked

A

is bronchoscopy likely to aid diagnosis

37
Q

is bronchoscopy likely to aid diagnosis? NO

A

surgical lung biopsy- if not excessive risk

- can make confident diagnosis

38
Q

is bronchoscopy likely to aid diagnosis? YES

A

Bronchoscopy

39
Q

Bronchospy 92)

A
  • BAL

- Endoscopic lung biopsy

40
Q

BAL fluid analysis

A
  • visual inspection
  • cell count
  • microbiology
  • malignant cell staining
41
Q

Endoscopic lung biopsy

A
  • histopathology
  • staining
  • immunohistochemistry
42
Q

diffusion capacity of the lungs (DLco)

A

Diffusing capacity of the lungs for carbon monoxide (DLCO) is a medical test that determines how much oxygen travels from the alveoli of the lungs to the blood stream.

43
Q

FEV1

A

The forced expiratory volume in one second (FEV1) measurement shows the amount of air a person can forcefully exhale in one second of the FVC test. (spirometer)

44
Q

FVC

A

Vital capacity is the maximum amount of air a person can expel from the lungs after a maximum inhalation. (spirometer)

45
Q

FEV1/FVC

A

It represents the proportion of a person’s vital capacity that they are able to expire in the first second of forced expiration to the full, forced vital capacity.

(6-10) Translational Medical Science (40 decks)

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