08.25 - Drugs for Restrictive Lung Disease (Sweatman) - Questions Flashcards
Drugs with clinical benefit in iPF
None yet
Advantage of Endothelin-1 Receptor Antagonists over Prostacyclins
Orally active
Proportion of patienst that respond to vasodilator challenge
15%
2 notable toxicities of Azathioprine
Neoplasia, Pancytopenia
Metabolism of PDE5 inhibitors
CYP
Metabolism of CCB’s
CYP3A4 substrates
End points of Vasodilator Challenge
Decr PAP and CO
CCB’s used in PAH
Diltiazem, Nifedipine, Amodipine
Where does Wegener’s typically occur
URT, Lungs, Kidneys
Plexiform lesions result in
proliferation of monoclonal endothelial cells, SM cells, and an accumulation of circulating cells –> Obstruction of flow
Pro-fibrogenic factors released by activated epithelium in iPF
TGF-B, PDGF
3 Notable Toxicities of Rituximab
HTN, Allergic -it is, Asthenia
PDE5 inhibitors are Not to be used in pateints taking
Organic Nitrates
Effect of Prostacyclin on vascular SM
Vasodilation and Anti-Proliferation
3 notable Glucocorticoid toxicities
Pancreatitis, Osteoporosis, DM
Most common cause of death in premature infants
IRDS
Drug class with efficacy in PAH and Systemic HTN
CCB’s
Route and toxicity of Epoprostenol
IV, Monitor Bleeding
Agents used for vasodilator challenge of pulmonary circulation
Epoprostenol, Adenosine, Inhaled NO
Route and 2 toxicities of Treprostinil
SC or IV; Bleeding, CYP
Route of Endothelin-1 Receptor Antagonists
Oral
Adverse effects of CCB’s
Hypotension, CYP3A4 Substrate
Goodpasture Syndrome
Type 2 hypersensitivity against alpha-3 chain of type 4 collagen
Route and toxicity of Iloprost
Inhaled, Hemoptysis
MOA of Azathioprine
DNA, RNA synthesis inhibitor –> Apoptosis of T Cells
Histologic sine qua non of patients with iPAH or heritable PAH
Plexiform Lesions
Disadvantage of Endothelin-1 Receptor Antagonists
Expensive, Teratogenic, Bosentan (liver, blood toxicities)
How to determine if CCB’s will work
Vasodilator Challenge
Glucocorticoids inhibitor production of __ and promote apopotosis of ___
IL-1, TNF; Mac’s, Dendritic Cells, T Cells
Not all patients respond to CCBs, some develop
potentially fatal hemodynamic decompensation
Most potent anti-inflammatory agents
Gluoccorticoids
Size of vessels affected in Granulomatosis w/ Polyangiitis
Small-medium
Effect of Endothelin on vascular SM
Vasconstriction and SM proliferation
2 notable toxicites of MTX
Pulmonary Fibrosis/Pneumonitis; Malignant Lymphoma
Most common adverse events with Ambrisentan
Edema and Headache
4 treatments for Wegeners
Labeled: Rituximab; Off-Labed: Azathioprine, Cyclophosphamide, Corticosteroids
Role of MTX in Sarcoidosis
Increase in Adenosine-Mediated Immunosuppression
2 notable toxicities of Cyclophosphamide
Bladder Cancer; Myelo- and Lympho-proliferative malignancies
General MOA of Prostanoids
Pulmonary Artery Vasodilation, Retard SM growth, Disrupt platelet aggregation
Most common adverse event in Sildenafil and Tadalafil?
Headache
Tx of Goodpastures
Plasmapharesis
Metabolism, Interactions of Bosentan and Ambrisentan
Liver, CYP - Don’t use in liver dysfunction
Goal of CCB therapy
Achieve NYHA 1 or 2 after 3-4 months; Only 50% of 15% achieve this
Responsivenss of Idiopathic Pulmonary Fibrosis to anti-inflammatory drugs
Little to none
Goodpasture is type __ hypersenstivity
2
Bottom Line for Prostanoids
Expensive and difficult to administer
MOA of Cyclophosphamide
Alkylating Agent; Suppression of B lymphocyte activity and Ig Secretion
Length of effect of Rituximab
6-9 months
Route of PDE5 inhibitors
Oral
Treatment of Sarcoidosis
Glucocorticoids, Methotrexate
