Idiopathic Pulmonary Fibrosis

Question 1

Parenchymal (intrinsic) restrictive lung diseases

Answer 1

Sarcoidosis
IPF
DIP (desquamative interstitial pneumonitis)
Pneumoconiosis
Drug/radiation induced 
Asbestosis

Question 2

Extraparenchymal (extrinsic) restrictive lung dieases

Answer 2

Neuromuscular

Chest Wall

Question 3

Describe the current theory of idiopathic pulmonary fibrosis

Answer 3

Trigger –> inflammation (aberrant activation of alveolar epithelium and form fibroblastic/myofibroblastic foci)

No apoptosis is occuring so there is an excess of cells

No lung compliance –> Decreased ventilation

Question 4

Presentation of IPF?

Answer 4

Gradual onset of > 6 mos with DOE and/or nonproductive cough

5% have no symptoms

Systemic symptoms: weight loss, low-grade fever, fatigue, myalgia, arthralgias

Question 5

To dx Idiopathic Pulmonary Fibrosis:

Answer 5

MUST GET COMPLETE HX

meds, social, occupational, expsoure

Question 6

What meds have been found to cause IPF?

Answer 6

Amiodarone
Bleomycin
Nitrofurantoin

Question 7

Physical exam findings:

Answer 7

Inspiratory crackles
Digital clubbing
Holosystolic tricuspid regurgitation murmur

Question 8

What is the overall treatment of idiopathic pulmonary fibrosis?

Answer 8

Manage complications and underlying conditions

STOP SMOKING