Intersitial Lung Disease

Question 1

what is sarcoidosis

Answer 1

non-caseating systemic granulomatous disease of unknown cause
type 4 hypersensitivity

Question 2

epidemiology

Answer 2

commonest in northern europe
usual onset aged 20-40
affects afrocarribeans more commonly and severely
less common in smokers

Question 3

how can sarcoidosis be classified

Answer 3

acute or chronic

Question 4

symptoms of acute sarcoidosis

Answer 4

erythema nodosum
bolateral hilar lymphadenopathy
arthritis
uveitis
fever

Question 5

symptoms of chronic sarcoidosis

Answer 5

lung infiltrates (alveolitis)
skin infiltrations
peripheral lymphadenopathy
hypercalcaemia
other organs- splenomegaly, renal, hepatitis, neurological, myocardial

Question 6

differntials

Answer 6

lymphoma, TB, carcinoma, fungal infection

Question 7

pulmonary symptoms/signs of sarcoid

Answer 7

abnormal CXR, bilareral hilar lymphadenopathy, fibrosis, infiltrates, dry cough, progressive dysponea, reduced excercise tolerance, chest pain

Question 8

non pulmonary signs/symptoms of sarcoidosis

Answer 8

lymphadenopathy, hepatomegaly, splenomegaly, uveitis, conjunctivitis, glaucoma, bells palsy, neuropathy, meningitis, SOL, erythema nodosum, lupus pernio, cardiomyopathy, arrythmia, hypercalcaemia, renal stones, pituitary dysfunction

Question 9

what is the diagnostic test of sarcoidosis

Answer 9

tissue biopsy showing non caseating granulomas

Question 10

result of pulmonary function tests in sarcoidosis

Answer 10

restrictive picture

Question 11

what blood test abnormalities may be present in sarcoidosis

Answer 11

angiotensin converting enzyme levels increased (not diagnositic, but act as disease activity marker)
raised calcium
raised inflammatory markers

Question 12

treatment of sarcoidosis

Answer 12

acute - self limiting, no treatment usually
chronic - oral steroids (40mg prednisolone OD 4-6 weeks, reduce dose over 1 year), immunosuppression (azathioprine, methotrexate, anti-TNF)

Question 13

prognosis of sarcoidosis

Answer 13

often relapses - monitor chest x ray and pulmonary function for several years after

Question 14

what are the indications for steroids in sarcoidosis

Answer 14

uveitis
parenchymal lung disease
hypercalcaemia
neuro/cardiac disease

Question 15

causes of bilateral hilar lymphadenopathy

Answer 15

TB
sarcoidosis
mycoplasma
organic dust diseases eg silivosis, berylliosis
EAA
histocytosis

Question 16

what is extrinsic allergic alveolitis

Answer 16

type II hypersensitivity reaction to antigen
‘hypersensitivity pneumonitis’
can be acute or chronic

Question 17

causes of EAA

Answer 17

thermophilic actinomycetes (farmers lung, malt workers, mushroom workers)
avian antigens (bird fanciers lung)
drugs (gold, bleomycin, sulphasalazine)
sugar workers lung

Question 18

clinical features of acute EAA

Answer 18

cough, breathless, myalgia, classically symptoms occur several hours after exposure

Question 19

signs of acute EAA

Answer 19

pyrexia (not always), crackles but NO WHEEZE, hypoxia

Question 20

features of chronic EAA

Answer 20

breathless, weight loss, exertional dyspnoea, type 1 resp failure, cor pulmonale, may get clubbing

Question 21

chest x ray in acute EAA

Answer 21

pulmonary infiltrates

Question 22

chest x ray in chronic EAA

Answer 22

fibrosis usually UPPER ZONES

Question 23

pulmonary function test results in EAA

Answer 23

restrictive picture (low FEV1 and FVC, high or normal ratio)
low gas transfer TLCO

Question 24

treatment of acute EAA

Answer 24

oxygen, steroid, antigen avoidance

Question 25

diagnosis of EAA

Answer 25

history of antigen exposure positive serum precipitins (IgG antibodies to the guilty antigen) can lung biopsy if in doubt

Question 26

treatment of chronic EAA

Answer 26

remove antigen exposure | oral steroids if breathless or low TCLO

Question 27

what is idiopathic pulmonary fibrosis

Answer 27

MOST common interstitial lung disease unknown cause progressive fibrosis

Question 28

pathology of IPF

Answer 28

'interstitial pneumonia'- cell infiltrate and inflammation results in fibrosis not an inflammatory disease ie. no hypersensitivity reaction

Question 29

signs/symptoms of IPL

Answer 29

several years - breathless, dry cough, malaise, weight loss, arthralgia cyanosis, clubbing, fine end expiratory crepitations

Question 30

complications of IPL

Answer 30

respiratory failure | increased risk of lung cancer

Question 31

Give other cause of pulmonary fibrosis

Answer 31

rheumatoid, SLE, systemic sclerosis, asbestos, medications

Question 32

name medications that can cause pulmonary fibrosis

Answer 32

amioderone, busulphan, bleomycin, nitrifuantoin, methotrexate

Question 33

CT scan findings in IPF

Answer 33

reticulonodular shadowing, worse at bases, broncietesis, honey combing cystic changes CT scan diagnostic can do lung biopsy if not

Question 34

treatment of IPF

Answer 34

steroids and immunosuppresion do not change disease course typically supportive - oxygen, new antifibrotic drugs - pirfenidone and nintedanib (expensive and many side effects) lung transplant if young

Question 35

prognosis of IPF

Answer 35

median survival 4 years from diagnosis