Restrictive Lung Diseases and Pulm Hypertension Treatment

Question 1

What is the treatment for pneumoconiosis?

Answer 1

There is no curative treatment for deposited material diseases such as silicosis, asbestosis or beryliosis. Patients should avoid further exposure.

Question 2

What are some pharmacological causes of ARDS?

Answer 2

excessive doses of aspirin, cocaine, opioids, phenothiazines, and antidepressants can all cause ARDS. Alcohol abuse increases risk, but can not cause ARDS itself.

Question 3

What is the treatment for the most common cause of respiratory failure in newborns?

Answer 3

NRDS is the most common cause and it is treated by giving the mother antenatal corticosteroids to increase fetal surfactant synthesis. If baby is born prematurely, exogenous surfactant (Poractant alfa, alfactant and beractant) are given.

Question 4

What is the hallmark sign of sarcoidosis?

Answer 4

non-caseating granulomas. Bilateral hilar lymphadenopathy

Question 5

How is sarcoidosis treated?

Answer 5

glucocorticoids or methotrexate

Question 6

What are the most potent anti-inflammatory agents and how do they work?

Answer 6

glucocorticoids. They inhibit production iof IL-1 and TNF while promoting IL-10. They also promote apoptosis of macors, dendritics and T cells.

Question 7

What are the significant adverse effects of chronic corticosteroid use?

Answer 7

HPA (hypothalamic-pituitary-adrenal) suppression. Osteoporosis, pancreatitis, diabetes mellitus, cataracts, glaucoma, psychosis, candidiasis, weight gain, immunosuppression

Question 8

What is the primary mechanism of methotrexate? How is it useful for sarcoidosis?

Answer 8

DHFR inhibition. It also increases adenosine-mediated immunosuppression, making it useful for treatment of sarcoidosis

Question 9

What are some of the significant adverse effects of methotrexate?

Answer 9

dermatologic reactions, birth deffects, lymphoma, risk of infection, fatal pulmonary effects (fibrosis, chronic interstitial pneumonitis)

Question 10

What is used to treat IPF (idopathic pulmonary fibrosis)?

Answer 10

IPF is NOT an inflammatory disease so antiinflammatory drugs will have no benefit. There are no drugs proven to be helpful.

Question 11

What type of hypersensitivity is Goodpasture syndrome? How is it treated?

Answer 11

Type II hypersensitivity against the a3 chain of type IV collagen in basement membranes of lungs and kidneys. Treat with plasmaphoresis to reduce autoantibody load.

Question 12

What is Wegener’s granulomatosis? How is it treated?

Answer 12

ANCA-positive autoimmune vascultis of lung and kidney. Treated with anti-inflammatory drugs like Rituximab, Azathioprine, and cyclophosphamide.

Question 13

What is the mechanism of Rituximab? What are some of its major side effects?

Answer 13

anti-CD20 antibody that depletes B-cells for 6-9 months after just 3 doses. 3 mechanisms: ADCC, complement activation, and apoptosis promotion

Major side effects: hypertension, asthenia, pruritis

Question 14

What is the mechanism of azathioprine? What are the major side effects?

Answer 14

DNA and RNA synthesis inhibitor that also produces immunosuppression by promoting apoptosis of T cells.
Risk of neoplasms, leukopenia and thrombocytopenia

Question 15

What is the mechanism of cyclophosphamide and what are its major toxicities?

Answer 15

an alkylating agent that depletes B and T cels and decreases Ig secretion.
NEutro and thrombocytopenia, bladder cancer, other malignancies

Question 16

What are the 4 causes of pulmonary hypertension (PHT)?

Answer 16

imbalance between vasoconstriciton and vasodilation, smooth muscle and endothelial cell proliferation, thrombosis, fibrosis

Question 17

What does prostacyclin do in relation to PHT?

Answer 17

inhibits platelet activation and smooth muscle growth. Also keeps thromboxane A2 levels down, reducing likelihood of platelet aggregation

Question 18

What does endothelin-1 do in relation to PHT?

Answer 18

induces smooth muscle cell proliferation.

Question 19

What does NO do in relation to PHT?

Answer 19

inhibits platelet stimulation and smooth muscle cell activity

Question 20

What are prostanoids?

Answer 20

drugs that induce pulmonary artery vasodilation, retard smooth muscle growth and disrupt platelet aggregation. They include Epoprostenol, Iloprost, and Treprostinil

Question 21

What are epoprostenol’s route and side effects?

Answer 21

prostanoid. Requires IV infusion and causes dose limiting hypotension, muscle pains, headaches and flushing

Question 22

What are iloprost’s route and side effects?

Answer 22

inhaled prostanoid. Can cause cough, flushing, tongue/back pains. Possible hemoptysis.

Question 23

What are treprostinil’s route and side effects?

Answer 23

continuous SC or IV infusion prostanoid. Can cause rash, headache, vasodilation, jaw pain and bleeding. CYP2C8 interactions (decreased clearance with gemfibrozil and increased with rifampin)

Question 24

What are Endothelin-1 receptor antagonists?

Answer 24

block the smooth muscle proliferation and pulmonary arterial vasoconstriciton produced by ETa (on smooth muscle) and ETb (on endothelial cells)

Question 25

What is the advantage of endothelin-1 receptor antagonists over prostacyclins?

Answer 25

endothelin-1 receptor antagonists are orally active, whereas prostacyclins must be IV or inhaled almost constantly. Downsde is they are teratogenis and bosentan can cause liver and blood toxicity

Question 26

What is bosentan and what are its major side effects?

Answer 26

endothlein-1 antagonist. Significantly elevated LFTs, anemia, extensive hepatic metabolism.

Question 27

What is ambrisentan and what are its major side effects?

Answer 27

endothelin-1 antagonist. Not likely to elevate LFTs like bosentan. Hepatically eliminated. Peripheral edema and headache.

Question 28

What are phosphodiesterase type 5 inhibitors? What is a serious contraindication?

Answer 28

they increase cGMP, causing vasodilation and reducing cellular proliferation. MUST NOT give with nitrates. Can be used to treat BPH and ED.

Question 29

What is tadalafil? Side effects?

Answer 29

phosphodiesterase type 5 inhibitor. Headache, changes in color vision.

Question 30

What is diltiazem and what are its major side effects?

Answer 30

CCB. can cause bradycardia, hypotension, headache and edema

Question 31

What is amlodipine and what are its major side effects?

Answer 31

CCB. Can cause edema, fatigue and hypotension

Question 32

MOA of verapamil? Adverse effects?

Answer 32

non-hydro CCB. Asystole, arrhythmias, etc.

Question 33

After what test can you give a patient CCB’s for PAH?

Answer 33

a vasodilator challenege of pulmonary ciculation – use epoprostenol, adenosine, and inhaled NO. If pts receive a carefully escalated dosing rate there is decreased PAP and CO then they can be given CCBs.