Drugs for Restrictive Lung disease

Question 1

Treatment options for ARDS

Answer 1

1. B-2 Agonist (albuterol) - vasodilate
2. Inhaled Nitric Oxide – vasodilate
3. Inhaled prostacycline – vasodilate
4. Corticosteroids – anti-inflammation
   Dietary oil supplements – anti-inflammation

Question 2

Treatment for NRDS

Answer 2

1. Antenatal corticosteroids for suspected Pre-matures
   a. Matures lungs more quickly
   Exogenous Surfactant (Poractant Alfa, Calfactant, and Beractant)

Question 3

Treatment for Sarcoidosis

Answer 3

a. Glucocorticoids - IL-1b, TNF; IL-10

b. Methotrexate (off-label) – adenosine-mediated immunosuppression (AMPDA, AMP, Adenosine, cAMP, immunosuppression)

Question 4

Treatment for Idiopathic pulmonary fibrosis

Answer 4

a. Pirfenidone – inhibitor of pulmonary fibroblast proliferation ; hepatic metabolism
b. Nintedanib – VEGFR/FGFR/PDGFR inhibitor (a TKR)
i. Primarily metabolized by esterases (minor CYP)

Question 5

Treatment for Goodpastures

Answer 5

Plasmapheresis

Question 6

Treatment for Wegners or polyangitis with granulmatosis

Answer 6

a. Rituximab – immunosuppressing MAB for CD20 on B precursors and B-Cells
i. Ab-dependent cell-mediated cytotoxicity
ii. Complement-mediated cytotoxicity
iii. Induction of apoptosis
iv. SIDE EFFECTS – HTN, Asthenia, Pruritis, Urticaria, Rhinitis, Arthralgia
b. Azathiprine (Off-label) – a DNA and RNA synthesis inhibitor; immunosuppressant (maybe via T cell apoptosis)
i. SIDE EFFECTS – Neoplastic, Mutagenic, leukopenic, and thrombocytopenic toxicity; Risk of Infection
c. Cyclophosphamide (Off-label) – alkylating agent producing B and T cell lymphopenia, B Lymphocyte suppression, IG secretion
i. SIDE EFFECTS – Neutro- and Thrombocytopenia, Bladder Cancer, Myeloproliferative/lymphoproliferative malignancies
d. Corticosteroids(Off-label)

Question 7

Treatment for PAH (plexiform lesions)

Answer 7

Prostanoids, Endothelin 1 receptor antagonist, PDE type 5 inhibitors, CCBs

Question 8

Treatment for CF

Answer 8

Ivacaftor, Ivacaftor/Lumacaftor, mucolytics

Question 9

What are the prostanoids

Answer 9

EpoPROSTenol (IV), IloPROST (6-9inhalations)(hemoptysis), TroPROSTinil(SC or IV, 2C8 interaction) (All induce pulmonary artery vasodilation, retard smooth muscle growth, and disrupt platelet aggregation)

Question 10

What are the Endothelin 1 receptor antagonists

Answer 10

Bosentan and Ambrosentan

Question 11

Bosentan and Ambrosentan are metabolized by what CYPs

Answer 11

CYP2C9 and 3A4 (bosentan also induces)

Question 12

What are the PDE5 inhibitors

Answer 12

Tadalafil (3A4 and 2C9) and Sildenafil (3A4)

Question 13

Ivacaftor and Lumacaftor inhibit what CYP

Answer 13

3A4

Question 14

Bosentan adverse rxns

Answer 14

significantly elevated LFTs, monitor ; anemia ; nasopharyngitis, headache ; extensive hepatic metabolism

Question 15

Amborsentan adverse rxns

Answer 15

peripheral edema and headache are most common

Question 16

Tadalafil adverse rxns

Answer 16

change in color vision (non-arteritic anterior ischemic optic neuropathy (NAION)

Question 17

Sildenafil adverse rxns

Answer 17

1. Headache most common ; epistaxis ; flushing, insomnia, dyspnea ; rarely – “a dizziness with sudden hearing loss”

Question 18

What CCBs are used in PAH

Answer 18

Diltiazem, nifedipine, amlodapine (all cyp 3A4)

Question 19

What mutation is Ivacaftor used with

Answer 19

G551D