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CR - Interstitial Lung Disease (i) (Hx (Hx of connective tissue disease…
CR - Interstitial Lung Disease (i)
intro
reticular pattern (net like)
heterogeneous group of diseases
fibrotic
disruption of distal lung parenchyma (gas exchange occurs here)
hallmark
extensive alteration of alveolar/airway architecture
scarring
thicker diffusion barrier - reduced oxygenation ability
gradual replacement of alveoli with fibrotic tissue
interstium (space between alv capill endothelial cells + alveolar septae/walls - contains lung connective tissue elements, becomes filled with fluid in CHF) filled with fibroblasts
restrictive
Classification
aetiological
idiopathic
IPF (slightly more common in men (60%)
sarcoidosis
Langerhan's cell histiocytosis (aka histiocytosis X)
collagen vasc disease (systemic)
RA
SLE
scleroderma
more common in women
Tx/drug-related
amiodarone (antiarrythmitic)
nitrofurantoin (antiUTI antibiotic)
MTX
bleomycin
radioTx (radiation pneumonitis post-tx for breast carcinoma or mediastinal lymphoma)
prolonged high dose O2
busulphan (alkylating agent - chemotx)
occupational/environmental exposures
inorganic
asbestosis
silicosis
organic
hypersensitivity pneumonitis (pigeon-fancier's / farmer's lung)
post -infectious (e.g. TB)
radiological
used most in clinical setting
based on zones most affected on CXR
upper = CHHARTS
CWP
hypersensitivity pneumonitis
histiocytosis X
ankylosing spondylitis (arthritis of spine)
radiation
TB
silicosis
lower = RAIDS
RA
asbestosis
IPF
drugs
scleroderma
histological
if tissue available
if more cellular it's more response to Tx (usually steroids or other immunosuppressant)
90% of cases don't get a Bx (histology inferred from clinical presentation + radiology)
usual interstitial pneumonitis (UIP) by far most common (90%)
IPF if no cause found
could be due to collagen vasc disorder
non-specific interstitial pneumonitis (NSIP)
v rare forms
desquamative interstitial pneumonitis (DIP)
resp bronchiolitis ILD (RB-ILD)
lymphocytic interstitial pneumonitis (LIP)
acute interstitial pneumonia (AIP)
bronchiolitis obliterans organising pneumonia (BOOP)
Hx
slowly progressive exertional dyspnoea
usually gradual (6-12+ months)
but sometimes can be acute/subacute (often misdxed as pneumonia)
dry cough (productive rare)
no wheeze (not an airway disease in its pure form, however COPD/asthma can coexist)
haemoptysis unusual - red flag so exclude...
bronchiectasis
malignancy (ILD patient's have x10 increased risk of getting cancer)
age
IPF > 60 y/o @ presentation
sarcoidosis + collagen vasc disorders 20-40 @ presentation
exposures
smoking associated with IPF + RB-ILD-> cured in 2 wks if patient quits!
drugs
OTC
protein supplements
radiotx
occupations
list in chronological order with duration for each
sandblasting (silica), coal mining, asbestos, hay or mushroom farming
hobbies/pets (birds-not just pigeons)
Hx of connective tissue disease may precede lung symptoms by months/years
musculoskeletal pain
weakness
fatigue
fever
joint pain/swelling
photosensitivity
pleuritis
dry eyes/mouth
scleroderma: tight skin over hands/ mouth/ nose, Raynaud's phenomenon (colour change with temp), dysphagia
BX via VATS (video-assisted thoracoscopic surgery)
not performed if there's a confident radiological Dx
performed if there's an atypical CXR or if there's progressive/unusual symptoms (fever, weightloss, vasculitis, haemoptysis)
risks
general anaesthetic
post-op chest drain needed