Interstitial Lung disease Flashcards
what are the two key symptoms of interstitial lung disease?
dyspnoea
dry cough
what medication can cause interstitial lung disease?
bleomycin busulfan amiodarone nitrofurantoin sulfasalazine methotrexate methysergide
other than medication what else can cause interstitial lung disease?
asbestosis, silicosis
hypersensivity pneumonitis
TB, viral, fungal infections
what systemic disease is interstitial lung disease associated with?
sarcoidosis RA SLE systemic sclerosis sjogrens MCTD (mixed connective tissue disease - lupus, scleroderma and polymyositis) UC ankylosing spondylitis
what causes upper lobe interstitial lung disease ?
Aspergillosis : ABPA Pneumoconiosis: Coal, Silica Hypersensitivity pneumonitis Negative, sero-arthropathy TB
what causes lower lobe interstitial lung disease ?
Sarcoidosis (mid zone) Medication Asbestosis Idiopathic pulmonary fibrosis Rheum: RA, SLE, Scleroderma, Sjogren’s, PM/DM
what type of hypersensivity reaction occurs in hypersensivity pneumonitis
Type 3 and 4
type 4 - granuloma formation and obliterative bronchiolitis
state three causes of hypersensivity pneumonitis ?
Bird fancier’s lung: proteins in bird droppings
Farmer’s / mushroom worker’s
Malt worker’s lung: Aspergillus clavatus
what is the acute presentation of hypersensivity pneumonitis ?
fever, dry cough, malaise, dyspnoea, crackles
what is the chronic presentation of hypersensivity pneumonitis ?
increasing dyspnoea
wt loss
Type 1 resp failure
cor pulmonale
what are 4 histopathy features of hypersensivity pneumonitis ?
upper zone disease
foamy histiocytes
bronchiolitis obliterans
soft centracinar epitheloid granuloma
what investigations should be done for hypersensivity pneumonitis ?
Bloods
CXR
spirometry (restrictive)
bronchoalveolar lavage
what would bronchoalveolar lavage show for hypersensivity pneumonitis ?
↑ lymphocytes and mast cells
what appearance will the lung have on CXR with hypersensivity pneumonitis ?
honeycomb lung
fibrosis
what is the medical treatment for hypersensivity pneumonitis ?
avoid exposure
steroids (acute/long term)
compensation may be payable (UK industrial injuries Act)
what is another name for idiopathic pulmonary fibrosis ?
cryptogenic fibrosing alveolitis
what age and gender is most affected by idiopathic pulmonary fibrosis ?
middle age
males
what is the commonest cause of interstitial lung disease?
idiopathic pulmonary fibrosis
what are the symptoms of idiopathic pulmonary fibrosis ?
dry cough dyspnoea maliase arthralgia obstructive sleep apnoea
what are the signs of idiopathic pulmonary fibrosis ?
cyanosis
clubbing
crackles (fine, end inspiratory)
what are the complications of idiopathic pulmonary fibrosis ?
increased risk of lung carcinoma
type 2 resp failure
cor pulmonale
what antibodies can be positive with idiopathic pulmonary fibrosis ?
ANA (anti nuclear antibody) -30%
RF (rheumatoid factor) -10%
would idiopathic pulmonary fibrosis have increased or decreased lung volume on CXR?
decreased lung volume due to fibrosis
what imaging can be done for idiopathic pulmonary fibrosis after CXR?
high resolution CT
what investigations can be done for idiopathic pulmonary fibrosis ?
bloods (CRP, ANA, RF, ABG) CXR HRCT spirometry lung biopsy bronchoalveolar lavage DTPA scan (may reflect disease activity)
what is the management for idiopathic pulmonary fibrosis ?
smoking cessation pulmonary rehabilitation O2 therapy anti-fibrotics (pirferidone, nitedanib) surgery - lung transplant (only cure)
what is 5YS for idiopathic pulmonary fibrosis ?
50%
state three industrial lung diseases?
Coal-workers Pneumoconiosis
Silicosis
Asbestosis
what work environment is associated with asbestosis ?
Demolition and ship building
what does asbestosis increase the risk of?
mesothelioma
what are the signs and symptoms of mesothelioma
Chest pain, wt. loss, clubbing, recurrent effusions, dyspnoea.
how is a diagnosis of mesothelioma made?
pleural biopsy
what is the average survival of asbestosis?
<2yrs
what work environment is associated with silicosis ?
Quarrying, sand-blasting
what does the CXR for Coal-workers Pneumoconiosis look like?
CXR: upper zone fibrotic masses
what does the CXR for silicosis look like?
Upper zone reticular shadowing and egg shell
calcification of hilar nodes → progressive massive fibrosis
what are 5 histopathological features of idiopathic pulmonary fibrosis ?
basal and posterior fibrosis
type 2 pneumocyte hyperplasia
proliferating fibroblastic foci
smooth muscle and vascular proliferation
evidence of new and old injury
what is the biopsy of idiopathic pulmonary fibrosis described as?
usual interstitial pneumonitis
What is the management of the industrial dust lung diseases? (Coal-workers Pneumoconiosis, silicosis and asbestosis)
avoid exposure
claim compensation from UK industrial injuries Act
which industrial lung diseases can have the complication of progressive massive fibrosis ?
Coal-workers Pneumoconiosis
silicosis
what are the three types of asbestosis ?
white (Chrysotile) - least fibrogenic
blue (Crocidolite) - most fibrogenic
brown (Amosite) - least common
what chemo can be used for malignant mesothelioma ?
pemetrexed and cisplatin
