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CR - Interstitial Lung Disease (iii) (Sarcoidosis (other organs commonly…
CR - Interstitial Lung Disease (iii)
IPF
poor prognosis
early Dx = NB
mean survival after Dx = 3-5 yrs (can be accelerated due to acute exacerbations)
pathogenesis theories
abnormally short telomeres in alveolar cells - accelerated lung ageing
basement membrane damage
oxidative stress
Tx
no effective cure
PPI (e.g. omeprazole) -> stops acid reflux being aspirated
antifibrotic drugs - pirfenidone, nintedanib (kinase inhibitor)
slow progress but can't halt it
NOT STEROIDS
non-responsive
don't use unless suspicious of NSIP (beware of SEs)
O2
pul rehab
transplant if under 65 (however age is not an absolute contraindication)
palliative measures
Sarcoidosis
systemic granulomatous disease
involves lungs in 90-95% of cases (incl lymphadenopathy)
60-80% remit spontaneously
other organs commonly involved
neurosarcoidosis (in aggressive forms)
heart (in aggressive forms)
conduction defects
risk of sudden death
sometimes patient needs pacemaker/ICD
liver (hepatic granulomas)
eyes (blindness risk)
skin
bone
blood
metabolic
more aggressive forms common in Afro-Caribbeans, not so much in ire (usually don't require Tx)
responds to immunosuppression (steroids, e.g. prednisolone)
fever, fatigue, weightloss, malaise in 30%
bihilar lymphadenopathy or other CXR sign in 25%
pul symptoms in 30-50%
Lofgren's syndrome = BHL + erythema nodosum
more acute
better prognosis
resolves faster
hypercalcaemia -> renal impairment
Dx
Hx + exam
CXR +/- HRCT
PFTs
renal, liver, bone profile (esp Ca)
eye exam
urinary Ca
tuberculin skin test
as dDx = TB (also has BHL, but has caseating granulomas instead on non-caseating)
must exclude other causes of granulomatous inflamm
infection (TB/fungal) - mantoux, cultures
foreign bodies (e.g. talcosis in IVDUs - contaminants deposit in lungs)
silicosis
berylliosis
hypersensitivity pneumonitis (birds, MAI complex from hot tubs)
lymphoma / carcinoma (do Bx)
SLE
who to Tx?
critical organ involvement (ocular, cardiac, neurological)
hypercalcaemia
progressive resp disease (observe over 3-6 months)
systemic illness
joint pain
cut symptoms
SURT - sarcoidosis of upper resp tract
Hypersensitivity pneumonitis
previously known as EAA-extrinsic allergic alveolitis
Ig in response to organic antigens
thermophilic actinomycetes
aspergillus
avian antigens (in bird droppings)
farmer's lung
pigeon-fancier's lung
type 3 hypersensitivity acutely
may progress to type 4 if chronic (pul fibrosis in upper lobes)
symptoms 6-8 hrs post-exposure
dry cough
SOB
chills
NO WHEEZE
acute basal creps
Dx
specific IgE
serum precipitins
clinical Dx: 98% likely if these 6 things are present...
antigen exposure (incl pillows)
+ve serum precipitins
recurrent symptomatic episodes
crackles
increased symptoms after exposure
weightloss
gold standard = HRCT + BAL (type 4 = lymphocytosis of>30% seen)
Tx
avoid allergen (mask)
give steroids if necessary (responsive)
good prognosis if Dxed early