08.25 - Drugs for Restrictive Lung Disease (Sweatman) - Questions Flashcards

1
Q

Drugs with clinical benefit in iPF

A

None yet

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1
Q

Advantage of Endothelin-1 Receptor Antagonists over Prostacyclins

A

Orally active

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2
Q

Proportion of patienst that respond to vasodilator challenge

A

15%

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3
Q

2 notable toxicities of Azathioprine

A

Neoplasia, Pancytopenia

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3
Q

Metabolism of PDE5 inhibitors

A

CYP

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3
Q

Metabolism of CCB’s

A

CYP3A4 substrates

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3
Q

End points of Vasodilator Challenge

A

Decr PAP and CO

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4
Q

CCB’s used in PAH

A

Diltiazem, Nifedipine, Amodipine

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5
Q

Where does Wegener’s typically occur

A

URT, Lungs, Kidneys

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5
Q

Plexiform lesions result in

A

proliferation of monoclonal endothelial cells, SM cells, and an accumulation of circulating cells –> Obstruction of flow

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7
Q

Pro-fibrogenic factors released by activated epithelium in iPF

A

TGF-B, PDGF

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7
Q

3 Notable Toxicities of Rituximab

A

HTN, Allergic -it is, Asthenia

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8
Q

PDE5 inhibitors are Not to be used in pateints taking

A

Organic Nitrates

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9
Q

Effect of Prostacyclin on vascular SM

A

Vasodilation and Anti-Proliferation

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10
Q

3 notable Glucocorticoid toxicities

A

Pancreatitis, Osteoporosis, DM

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12
Q

Most common cause of death in premature infants

A

IRDS

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13
Q

Drug class with efficacy in PAH and Systemic HTN

A

CCB’s

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13
Q

Route and toxicity of Epoprostenol

A

IV, Monitor Bleeding

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13
Q

Agents used for vasodilator challenge of pulmonary circulation

A

Epoprostenol, Adenosine, Inhaled NO

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14
Q

Route and 2 toxicities of Treprostinil

A

SC or IV; Bleeding, CYP

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14
Q

Route of Endothelin-1 Receptor Antagonists

15
Q

Adverse effects of CCB’s

A

Hypotension, CYP3A4 Substrate

17
Q

Goodpasture Syndrome

A

Type 2 hypersensitivity against alpha-3 chain of type 4 collagen

18
Q

Route and toxicity of Iloprost

A

Inhaled, Hemoptysis

20
MOA of Azathioprine
DNA, RNA synthesis inhibitor --\> Apoptosis of T Cells
21
Histologic sine qua non of patients with iPAH or heritable PAH
Plexiform Lesions
21
Disadvantage of Endothelin-1 Receptor Antagonists
Expensive, Teratogenic, Bosentan (liver, blood toxicities)
21
How to determine if CCB's will work
Vasodilator Challenge
23
Glucocorticoids inhibitor production of __ and promote apopotosis of \_\_\_
IL-1, TNF; Mac's, Dendritic Cells, T Cells
24
Not all patients respond to CCBs, some develop
potentially fatal hemodynamic decompensation
26
Most potent anti-inflammatory agents
Gluoccorticoids
28
Size of vessels affected in Granulomatosis w/ Polyangiitis
Small-medium
30
Effect of Endothelin on vascular SM
Vasconstriction and SM proliferation
32
2 notable toxicites of MTX
Pulmonary Fibrosis/Pneumonitis; Malignant Lymphoma
32
Most common adverse events with Ambrisentan
Edema and Headache
33
4 treatments for Wegeners
Labeled: Rituximab; Off-Labed: Azathioprine, Cyclophosphamide, Corticosteroids
34
Role of MTX in Sarcoidosis
Increase in Adenosine-Mediated Immunosuppression
36
2 notable toxicities of Cyclophosphamide
Bladder Cancer; Myelo- and Lympho-proliferative malignancies
37
General MOA of Prostanoids
Pulmonary Artery Vasodilation, Retard SM growth, Disrupt platelet aggregation
38
Most common adverse event in Sildenafil and Tadalafil?
Headache
40
Tx of Goodpastures
Plasmapharesis
41
Metabolism, Interactions of Bosentan and Ambrisentan
Liver, CYP - Don't use in liver dysfunction
42
Goal of CCB therapy
Achieve NYHA 1 or 2 after 3-4 months; Only 50% of 15% achieve this
43
Responsivenss of Idiopathic Pulmonary Fibrosis to anti-inflammatory drugs
Little to none
44
Goodpasture is type __ hypersenstivity
2
46
Bottom Line for Prostanoids
Expensive and difficult to administer
47
MOA of Cyclophosphamide
Alkylating Agent; Suppression of B lymphocyte activity and Ig Secretion
48
Length of effect of Rituximab
6-9 months
49
Route of PDE5 inhibitors
Oral
50
Treatment of Sarcoidosis
Glucocorticoids, Methotrexate