Restrictive Lung Disease + IPF

Question 1

What restrictive lung disease?

Answer 1

Characterized by either a reduction in lung compliance or increased external pressures around the lungs, limiting lung inflation during inhalation

Question 2

What are external etiologies of restrictive lung disease?

Answer 2

Fluid
Masses
Abnormal thoracic anatomy

Question 3

What are intrinsic etiologies of restrictive lung disease?

Answer 3

Loss of lung compliance due to pathological lung parenchymal changes
-thickening or fibrosis of alveolar tissues
-lungs become stiff

Question 4

What would a PFT of restrictive lung disease look like?

Answer 4

TLC decreased
FEVI decreased
FVC decreased
FEVI/FVC 2
- Normal: the FVC and FEVI are equally effected
- Increased: FVC reduction is larger than FEVI

Question 5

What is acute restrictive lung disease?

Answer 5

It leads to sudden respiratory distress and potentially failure
Sudden influx of fluid into alveolar spaces and tissues (edema)

ARDS- acute respiratory distress syndrome.

Sudden, massive collection of fluid in the pleural space
Hemothorax or pleural effusion

Question 6

What is chronic restrictive lung disease?

Answer 6

ILD Interstitial lung disease

Question 7

What is idiopathic pulmonary fibrosis IPF?

Answer 7

It’s a type of IIP- idiopathic interstitial pneumonia

Restrictive lung disease that results in diffuse lung scarring (fibrosis)

Question 8

IPF =

Answer 8

UIP Usual Interstitial pneumonia

Question 9

How common is IPF?

Answer 9

The most common type of idiopathic interstitial pneumonia

Question 10

Although idiopathic, what do we think is the pathology for IPF

Answer 10

Likely due to recurrent cycles of epithelial cell injury and dysregulated repair

Question 11

What factors are associated with IPF?

Answer 11

Smoking
Autoimmune/rheumatic disease
Long term exposure to irritant fumes/dust particles

Question 12

How does IPF present clinically?

Answer 12

Dry Cough
Progressive dyspnea
(TOTALLY ISOLATED TO LUNGS)

Question 13

What kind of lab do you run to check for IPF?

Answer 13

Rhematological work up to rule out autoimmune etiology

Question 14

What would you see in spirometry of IPF?

Answer 14

Restrictive
TLC Decreased
FEVI Decreased
FVC
FEVI/FVC Normal or increased

Question 15

What would you see on chest x-ray of IPF?

Answer 15

Increased reticular markings
“Ground glass” opacities***

Question 16

What would you see on chest CT scan of IPF?

Answer 16

Increased reticular opacities and honeycomb-type changes
Ground glass opacities

Question 17

Do we do a lung biopsy on everyone?

Answer 17

It is the gold standard for definitive diagnosis but its only necessary for atypical cases.

Question 18

How do you treat IPF?

Answer 18

Supplement O2
-Initially only with exertion but later on continuously
- helps slow the progression of PHTN

Question 19

What medications can you give a patient with IPF?

Answer 19

Immune modulating medications being tested by FDA
-Nintendanib and pirfeniodone

Question 20

What do immune modulating medications do in IPF?

Answer 20

Slows the progression of cellular fibrosis, does not reverse fibrosis.

Question 21

What is the only definitive treatment for IPF?

Answer 21

Lung transplant

Question 22

What is the survival rate with lung transplant?

Answer 22

50% 5 year survival rate