ILD Flashcards
What will you find in BAL for Sarcoid?
CD4+/CD8+ ratio of more than 4
What is stage 2 sarcoid ?
LN and lung involvement
What is the median survival for people with IPF in the UK?
3 years from diagnosis (though is variable as 20% survives more than 5 years)
What are the clinical features of IPF?
Gradual onset exertional breathlessness and cough (often 9/12 prior to presentation)
What is estimated prevalence in the UK of IPF?
30,000 in UK
What is the purported cause of IPF?
Repeated alveolar epithelial injury leading to aberrant wound healing. Triggers include inhalation of metal/wood/GORD
What are the criteria for lung transplant in IPF?
TLCO <40%
FVC fall 10% over 6 months
TLCO fall 15% over 6 months
O2 desat to <88% on 6MWT
Should we ventilate patients with IPF?
Poor outcomes , do not routinely offer mechanical ventilation to patients with IPF who develop life threatening resp failure
What are the treatment guidelines for Pirfenidone in IPF?
FVC 50-80%
dose is 267mgTDS
What are the treatment guidelines for Nintedanib in IPF?
FVC > 50%
(Due to Inbuild trial can now give you FVC >80%, lower cut off still in place)
What are the main side effects of Pirfenidone?
Nausea
Photosensitive rash
(NB can cause Liver Function derangement)
What are the main side effects of Nintedanib
Diarrhoea
Weight loss
Incr bleeding risk
(NB can cause liver function derrangement)
How is IPF histologically characterised?
Temporal and special heterogeneity with areas of active fibroblastic foci (reflecting acute injury) and architectural distortion / honeycombing (reflecting chronic inflammation) interspersed with areas of normal lung. Reflecting different stages of evolution of disease process across the lung
IPF is seen as the archetypal ILD
What lung diseases associated with a UIP pattern
IPF
CTD-ILD
Chronic HP
Sarcoid
Nitrofurantoin ILD
Asbestosis
Who gets IPF?
Men
60-70 classically
Ex smokers often
What tests to do in IPF?
Bloods : ANA, ANCA, RhF, Anti CCP, Immunoglobulins, HIV, ?Avian precipitans ?Polymositis scleroderma panel (ie ruling out other causes). ESR and CRP often mildly raised , mild anaemia
NB can get positive RhF and / or ANA at low titres
Lung function
HRCT
?BAL
?Biopsy (rarely done)
TTE
What is Nintedanib
Tyrosine Kinase Inhibitor (TKI) which inhibits platelet derived growth factor (PDGFR) , fibroblast growth factor (FGFR) and vascular endothelial growth factor receptor (VEGFR)
Combined analysis of three nitedanib trials has shown that it improves all cause mortality by 30% and respiratory related mortality by 38%
What is Pirfenidone
Unclear exact mechanism but inhibits collagen synthesis and reduces fibroblast proliferation
What signifies a failure of treatment with anti fibrotic?
FVC decline >10% in 6 months / 1 year or TLCO decline >15% in 6 months/1 year
What are poor prognostic factors in IPF?
Low BMI
TLCO <40% at presentation
FVC fall > 10% in 6 months
TLCO fall > 15% in 6 months
Pulmonary hypertension
Fibroblastic foci on biopsy
What do patients either IPF usually die of ?
Respiratory failure and infection
NB increased risk of lung cancer
What is inpatient mortality for IPF exacerbation ?
60%
What is a UIP picture on scan?
Basal/subpleural preponderance of reticulation
Traction bronchiectasis
Honeycombing
Propellar blade
With ABSENCE of :
- XS ground glass
- Bronchocentricity
- Lots of interlobular septal thickening
What is honeycombing
Multilevel, walled , 3-10 mm size
Beware
Emphysema with any sort of infiltration surrounding it can look like honeycombing , traction bronchiectasis can etc