Rx for Restrictive Lung Disease, PAH, and CF- Sweatman

Question 1

Treatment of Neonatal Respiratory Distress Syndrome?

Answer 1

Administration of antenatal corticosteroids (dexamethasone and betamethasone) to all women at risk of delivery less than 34 weeks

Question 2

What do all the surfactant drugs end in?

and name them (3)

Answer 2

-actant; Poractant alfa, Calfactant, Beractant –> given to neonats that don’t produce enough surfactant

Question 3

Treatment of sacroidosis?

Answer 3

Glucocorticoids and Methotrexate

Question 4

How does methotrexate help with immunosuppression?

Answer 4

Increase in adenosine-mediated immunosuppression

Question 5

Is Methotrexate front line therapy for sarcoidosis?

Answer 5

No; too many adverse effects

Question 6

What is the treatment for Idiopathic Pulmonary Fibrosis?

Answer 6

There is none –> don’t use anti-inflammatories

Question 7

What Rx are used in the treatment of Wegner’s granulomatosis? Which is labeled for this purpose?

Answer 7

Rituximab (labeled; anti-CD20), Azathioprine (DNA/RNA synthesis inhibitor), Cyclophosphamide (alkylating agent), and Glucocorticoids (inflammation and shit)

Question 8

Which class of Rx are the only drugs used for systemic arterial HTN that are also useful in treating pulmonary arterial hypertension?

Answer 8

Calcium channel blockers

Question 9

Which calcium channel blockers are used in treatment of pulmonary arterial hypertension?

Answer 9

Diltiazem, Nifedipine ER, Amlodipine

Question 10

Why is Verapamil not used to treat pulmonary arterial hypertension?

Answer 10

Depresses cardiac function too much

strong negative inotropic effects - more likely to produce symptomatic bradycardia than the others

Question 11

Name the Prostanoids

Answer 11

Epoprostenol, Iloprost, Trepostinil

Question 12

Which prostanoid causes hemoptysis?

Answer 12

Iloprost

Question 13

What is the most common ADE associated will all prostanoids?

Answer 13

Bleeding; patients need to be monitored, especially if on anticoagulants

Question 14

Administration of prostanoids?

Answer 14

IV (epoprostenol), inhaled (iloprost), SC (trepostinil)

NOT oral

Question 15

Name the Endothelin-1 Receptor antagonists

Answer 15

Bosentan and Ambrisentan –> both are teratogenic

Block SMC proliferation and pulmonary arterial vasoconstriction

Question 16

Unique ADE of Bosentan?

Answer 16

Significant liver toxicity; need to monitor LFTs

Question 17

What medication can you not take Type V phosphodiesterase inhibitors (boner pills) with?

Answer 17

“Do not take Viagra if you are currently taking Nitrates for chest pain”
“if you have an erection lasting for more than 4 hours, you should go to the hospital”
Listen, if I have a hard on for more than 4 hours, it’s my girlfriend that will need to go to the hospital

Question 18

Do all patients with pulmonary arterial hypertension respond to calcium channel blockers?

Answer 18

No; some even have life threatening circulatory collapse –> use vasodilator challenge before prescribing

Question 19

Which CF patients can you not use Ivacaftor on?

Answer 19

Those homozygous for F508del;

90% of CF patients have at least one copy of the DeltaF508 mutation

Question 20

How does Ivacaftor work?

Answer 20

improves chloride transport through mutated channels

Question 21

What is the effect of combining Ivacaftor and Lumacaftor?

Answer 21

Lumacaftor is a conformation stabilizer drug; end result is delivery of a fully functional protein to the apical surface of the cell and potentiation of that channel’s effect

Question 22

Pregnancy category of the Endothelin-1 Receptor antagonists?

Answer 22

Bosentan and Ambrisentan

Category X; severely teratogenic

Question 23

What CF patients does Ivacaftor work on? what deletion?

Answer 23

G551D mutation

Question 24

what 2 drugs are used for CF?

Answer 24

Ivacaftor and Lumacaftor

Question 25

What drugs can induce ARDS?

Answer 25

Aspirin, cocaine, opioids, phenothiazines (promethazine), tricyclic antidepressants (amitriptyline)

Question 26

What si the treatment for Goodpasture’s syndrome?

Answer 26

Plasmapheresis to remove anti-GBM antibodies