Important Notes on Restrictive Lung Disease

Question 1

What methods of treatment are used in ARDS?

Answer 1

• ß2-agonists, Glucocorticoids, etc.

**No real treatment, we just do these things hoping it will work

Question 2

What methods of treatment are there for NRDS?

Answer 2

Prophylactically: give antenatal corticosteriods if you think the baby will be born under 34 wks gestation

Treatment: Surfactant (Poractant, Calfactant, Bractant)

Question 3

What methods of treatment are there for Sarcoidosis?

Answer 3

• Glucocorticoids
• OR Methotrexate

**Note that MTX is NOT 1st line therapy due to risk of pulmonary fibrosis, and teratogenic effects

Question 4

What methods of treatment are there for IPF?

Answer 4

• Ninetdinib is the most effective tx. It blocks VEGF-R, FGF-R, and PDGF-R.
• Pirfenidone is thought to work too but we don’t know the MOA

Question 5

What method of treatment is there for Goodpasture’s?

Answer 5

Plasmapheresis

Question 6

What methods of treatment are there for patients with Wegner’s?

Answer 6

• Rituximab

or Azathioprine, Cyclophosphamide, or Corticosteriods

Question 7

What 4 methods of treatment are there for PAH?

• disadvantages to each?

Answer 7

Prostacyclin Analogues
• Iloprost => Hemoptysis
• Treprostenol => Jaw Pain

Endothelin-1 Anatagonist
• Bosentan => anemia, elevated LFTs
• Ambrisentan => peripheral edema
**Neither can be given to ppl. with liver dysfunction, and BOTH are teratogenic

PDE-5 inhibitors
•Sildenafil => Epistaxis, Hearing Loss
• Tadalafil => Color Vision Changes

CCB’s
• not effective in most pts.