Trigger 6: IPF Flashcards
incidence of IPF
is increasing
idiopathic pulmonary fibrosis is
a type of interstitial lung disease
pulmonary fibrosis is the ..
end stage of a heterogeneous group of interstitial lung disease
which interstitial lung disease is the most common
IPF
how many deaths per year in the UK
> 5,000
how may patients in the EU with IPf
360,000
median suvrival
2-3 years
how many active IPF trial
> 200
most common age to get IPF
70-75
currently no treatments which
cure or reverse scarring
causes of IPF
occurs when alveoli get damaged and increasingly scarred
although idiopathic, IPF is linked to
o dust, wood or metal exposure o viral infection o family history of IPF o Gastro-oesophageal reflux disease o smoking
pathological characterisation
- 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
basic mechanism of IPF
- 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
features of IPF
- scarring- honeycombing
- loss of elasticity
- inflammation
- loss of air filled spaces
- reduction in surface area
thoracic imaging shows
pleural surface has a cobblestone appearance.
on cross section
cobblestone reatino show areas of airspace enlargement and fibrotic retraction
airspace enlargement and fibrotic retraction
honeycombing
symptoms of IPF
- Shortness of breath
- Coughing
- Fever
- Weight loss
IPF onset
rapid
physical examination
- listen to the lungs
- looking at fingers
what will the lungs sound like
- lunk crackles ( IPF and asbestos lungs)
- pleural rub
what do fingers look like
clubbed
which pulmonary function tests can be done
o Forced vital capacity o Diffusion capacity of lung for CO (DLco) o FEV1/FVC ratio o Oxyhaemoglobin stas o 6-minute walk test
blood test
no specific blood test available
why is it important that patients receive an accurate diagnosis
to ensure correct treatment and management decision
diagnoses often invole
invasive and non invasive diagnostic procedures
Non-invasive diagnostic procedure
secured by the presence of UIP (usual interstitial pneumonia)- using High resolution computed tomography (HRCT)
what will HRCT show
- reticular lines
- traction bronchiectasis
evaluation of honeycombing shown on HRCT has been found vulnerable to
significant inter observer variability
diagnosis can sometime be made just using
high resolution computed tomography (HRCT)
name some invasive diagnostic procedure
bronchoscopy
bronchopleural laval (BAL)
lung biopsy
BAL is
analysed for infection or malignancies
10% of patients cannot undergo surgical biopsy due to
AE and advanced disease
diagnosis pathway
1) patient with suspected ILD
2) Comprehensive history, physical exam and appropriate lab testing
3) Thoracic imaging (Chest-xray, HRCT)
4) intermediate diagnosis
after intermediate diagnosis what question is asked
is bronchoscopy likely to aid diagnosis
is bronchoscopy likely to aid diagnosis? NO
surgical lung biopsy- if not excessive risk
- can make confident diagnosis
is bronchoscopy likely to aid diagnosis? YES
Bronchoscopy
Bronchospy 92)
- BAL
- Endoscopic lung biopsy
BAL fluid analysis
- visual inspection
- cell count
- microbiology
- malignant cell staining
Endoscopic lung biopsy
- histopathology
- staining
- immunohistochemistry
diffusion capacity of the lungs (DLco)
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.
FEV1
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)
FVC
Vital capacity is the maximum amount of air a person can expel from the lungs after a maximum inhalation. (spirometer)
FEV1/FVC
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.
