Intersitial Lung Disease Flashcards

1
Q

what is sarcoidosis

A

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

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2
Q

epidemiology

A

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

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3
Q

how can sarcoidosis be classified

A

acute or chronic

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4
Q

symptoms of acute sarcoidosis

A
erythema nodosum
bolateral hilar lymphadenopathy
arthritis
uveitis
fever
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5
Q

symptoms of chronic sarcoidosis

A
lung infiltrates (alveolitis)
skin infiltrations
peripheral lymphadenopathy
hypercalcaemia
other organs- splenomegaly, renal, hepatitis, neurological, myocardial
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6
Q

differntials

A

lymphoma, TB, carcinoma, fungal infection

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7
Q

pulmonary symptoms/signs of sarcoid

A

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

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8
Q

non pulmonary signs/symptoms of sarcoidosis

A

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

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9
Q

what is the diagnostic test of sarcoidosis

A

tissue biopsy showing non caseating granulomas

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10
Q

result of pulmonary function tests in sarcoidosis

A

restrictive picture

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11
Q

what blood test abnormalities may be present in sarcoidosis

A

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

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12
Q

treatment of sarcoidosis

A

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)

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13
Q

prognosis of sarcoidosis

A

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

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14
Q

what are the indications for steroids in sarcoidosis

A

uveitis
parenchymal lung disease
hypercalcaemia
neuro/cardiac disease

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15
Q

causes of bilateral hilar lymphadenopathy

A
TB
sarcoidosis
mycoplasma
organic dust diseases eg silivosis, berylliosis
EAA
histocytosis
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16
Q

what is extrinsic allergic alveolitis

A

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

17
Q

causes of EAA

A

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

18
Q

clinical features of acute EAA

A

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

19
Q

signs of acute EAA

A

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

20
Q

features of chronic EAA

A

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

21
Q

chest x ray in acute EAA

A

pulmonary infiltrates

22
Q

chest x ray in chronic EAA

A

fibrosis usually UPPER ZONES

23
Q

pulmonary function test results in EAA

A
restrictive picture (low FEV1 and FVC, high or normal ratio)
low gas transfer TLCO
24
Q

treatment of acute EAA

A

oxygen, steroid, antigen avoidance

25
Q

diagnosis of EAA

A

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

26
Q

treatment of chronic EAA

A

remove antigen exposure

oral steroids if breathless or low TCLO

27
Q

what is idiopathic pulmonary fibrosis

A

MOST common interstitial lung disease
unknown cause
progressive fibrosis

28
Q

pathology of IPF

A

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

29
Q

signs/symptoms of IPL

A

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

30
Q

complications of IPL

A

respiratory failure

increased risk of lung cancer

31
Q

Give other cause of pulmonary fibrosis

A

rheumatoid, SLE, systemic sclerosis, asbestos, medications

32
Q

name medications that can cause pulmonary fibrosis

A

amioderone, busulphan, bleomycin, nitrifuantoin, methotrexate

33
Q

CT scan findings in IPF

A

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

34
Q

treatment of IPF

A

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

35
Q

prognosis of IPF

A

median survival 4 years from diagnosis