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

A

Oral

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
Q

MOA of Azathioprine

A

DNA, RNA synthesis inhibitor –> Apoptosis of T Cells

21
Q

Histologic sine qua non of patients with iPAH or heritable PAH

A

Plexiform Lesions

21
Q

Disadvantage of Endothelin-1 Receptor Antagonists

A

Expensive, Teratogenic, Bosentan (liver, blood toxicities)

21
Q

How to determine if CCB’s will work

A

Vasodilator Challenge

23
Q

Glucocorticoids inhibitor production of __ and promote apopotosis of ___

A

IL-1, TNF; Mac’s, Dendritic Cells, T Cells

24
Q

Not all patients respond to CCBs, some develop

A

potentially fatal hemodynamic decompensation

26
Q

Most potent anti-inflammatory agents

A

Gluoccorticoids

28
Q

Size of vessels affected in Granulomatosis w/ Polyangiitis

A

Small-medium

30
Q

Effect of Endothelin on vascular SM

A

Vasconstriction and SM proliferation

32
Q

2 notable toxicites of MTX

A

Pulmonary Fibrosis/Pneumonitis; Malignant Lymphoma

32
Q

Most common adverse events with Ambrisentan

A

Edema and Headache

33
Q

4 treatments for Wegeners

A

Labeled: Rituximab; Off-Labed: Azathioprine, Cyclophosphamide, Corticosteroids

34
Q

Role of MTX in Sarcoidosis

A

Increase in Adenosine-Mediated Immunosuppression

36
Q

2 notable toxicities of Cyclophosphamide

A

Bladder Cancer; Myelo- and Lympho-proliferative malignancies

37
Q

General MOA of Prostanoids

A

Pulmonary Artery Vasodilation, Retard SM growth, Disrupt platelet aggregation

38
Q

Most common adverse event in Sildenafil and Tadalafil?

A

Headache

40
Q

Tx of Goodpastures

A

Plasmapharesis

41
Q

Metabolism, Interactions of Bosentan and Ambrisentan

A

Liver, CYP - Don’t use in liver dysfunction

42
Q

Goal of CCB therapy

A

Achieve NYHA 1 or 2 after 3-4 months; Only 50% of 15% achieve this

43
Q

Responsivenss of Idiopathic Pulmonary Fibrosis to anti-inflammatory drugs

A

Little to none

44
Q

Goodpasture is type __ hypersenstivity

A

2

46
Q

Bottom Line for Prostanoids

A

Expensive and difficult to administer

47
Q

MOA of Cyclophosphamide

A

Alkylating Agent; Suppression of B lymphocyte activity and Ig Secretion

48
Q

Length of effect of Rituximab

A

6-9 months

49
Q

Route of PDE5 inhibitors

A

Oral

50
Q

Treatment of Sarcoidosis

A

Glucocorticoids, Methotrexate