idiopathic pulmonary fibrosis

Question 1

what is the epidemiology of IPF?

Answer 1

most common cause of iLD
middle aged
M>F 2:1
associated with other autoimmune disease in 30%

Question 2

what are the symptoms of IPF?

Answer 2

dry cough
dyspnoea
malaise
weight loss
arthralgia
obstructive sleep apnoeaw

Question 3

what are the signs of IPF?

Answer 3

cyanosis
clubbing
find end-inspiratory crackles

Question 4

what are the complications of IPF?

Answer 4

increase risk of lung ca
type 2 respiratory failure + cor pulmonale

Question 5

what do bloods show in IPF?

Answer 5

high CRP
high Ig
ANA+ 30%
RF+ 10%
ABG- hypoxic, hypercapnic

Question 6

what are CXR findings in IPF?

Answer 6

low lung volume
bilateral lower zone reticulonodular shadowing
honeycomb lung

HRCT more sensitive

Question 7

what are the spirometry findings for IPF?

Answer 7

restrictive
decreased transfer factor TLCO

Question 8

what are the bronchoalveolar lavage findings in IPF?

Answer 8

high lymphocytes- good prognosis
high PMN (polymorphonuclear neutrophils) or eosiniphils- bad prognosis

lung biopsy will show usual interstitial pneumonia

Question 9

what is the management of IPF?

Answer 9

supportive care- stop smoking, pulm rehab, o2, palliation, treat symptoms of heart failure

lung transplant is only cure

Question 10

what is the prognosis of IPF?

Answer 10

50% 5 year survival
average life expectancy is around 3-4 years